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Belfeki N, Kammoun S, Ghriss N, Eldirani C, Mekinian A. Current concepts on pathogenesis, diagnosis and management of hepatic sarcoidosis. Rheumatol Int 2025; 45:130. [PMID: 40304755 DOI: 10.1007/s00296-025-05888-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2025] [Accepted: 04/10/2025] [Indexed: 05/02/2025]
Abstract
Sarcoidosis is an inflammatory multisystemic granulomatosis of unknown cause, with a wide range of clinical features, characterized by the presence of non-caseating granulomas. Liver is the third involved organ after lungs and lymph nodes, with a reported prevalence of hepatic involvement in 5 to 25% of systemic symptomatic sarcoidosis. The immunopathogenesis of sarcoidosis is still unknown but it seems to involve the innate and adaptive immune actors in response to a putative antigen in genetically predisposed individuals. Because of its paucisymptomatic presentation, hepatic sarcoidosis may be underdiagnosed. Unspecified impaired general condition, fever, abdominal pain, and jaundice are the main symptoms associated with liver sarcoidosis. Frequently, laboratory liver tests are abnormal. Imaging tools may reveal liver nodular enlargement but can be totally normal. Liver biopsy is often required to confirm the diagnosis. A meticulous workup is mandatory to rule out differential diagnosis of hepatic granuloma. Portal hypertension and liver cirrhosis are the most prevalent complications of hepatic sarcoidosis. Treatment is not necessary in all cases and first line treatment in symptomatic patients requires corticosteroids and/or ursodeoxycholic acid. Immunosuppressants and biologics could be used as second line agents. In severe cases, liver transplantation is indicated.
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Affiliation(s)
- Nabil Belfeki
- Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud-Ile de France, 270 Avenue Marc Jacquet, Melun, 77 000, France.
| | - Sonia Kammoun
- Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud-Ile de France, 270 Avenue Marc Jacquet, Melun, 77 000, France
| | - Nouha Ghriss
- Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Sud-Ile de France, 270 Avenue Marc Jacquet, Melun, 77 000, France
| | - Charif Eldirani
- Departement of Gastroenterology and Hepatology, Groupe Hospitalier Sud-Ile de France, Melun, France
| | - Arsene Mekinian
- Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy, Assistance Publique - Hôpitaux de Paris, Hôpital Saint-Antoine Paris, Paris, France
- Sorbonne University, UMPC University Paris 06, INSERM U938, Centre de Recherche Saint- Antoine (CRSA), Paris, France
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2
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Semba S, Teraoka Y, Kamada H, Kato N, Mizumoto T, Tamaru Y, Hatakeyama T, Yamaguchi A, Kouno H, Yoshida S. A case of chronic hepatitis B complicated by systemic sarcoidosis that regressed following nucleos(t)ide analog therapy. Clin J Gastroenterol 2025:10.1007/s12328-025-02105-2. [PMID: 40009126 DOI: 10.1007/s12328-025-02105-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/19/2024] [Accepted: 02/10/2025] [Indexed: 02/27/2025]
Abstract
Herein, we report the case of a 75-year-old woman with systemic sarcoidosis complicated by chronic hepatitis B. She presented with high fever and fatigue, with imaging studies revealing multiple tumor nodules in the liver and spleen, along with generalized lymphadenopathy, leading to her admission to our hospital for further evaluation and treatment. Blood tests revealed chronic hepatitis B, and nucleoside analog therapy with entecavir was initiated before the sarcoidosis diagnosis. A liver biopsy confirmed sarcoidosis, and treatment continued without steroid use. Significant improvement in symptoms and tumor size reduction were observed within weeks. After 6 months of treatment, positron emission tomography/computed tomography showed further regression of the liver and spleen tumors, with no symptom recurrence. Given the rarity of sarcoidosis complicated by chronic hepatitis B, this case suggests that controlling hepatitis B virus infection with nucleos(t)ide analogs might improve sarcoidosis symptoms without steroid use. The immunologic mechanisms underlying this cooccurrence remain unclear, and such cases are extremely rare. Therefore, this case may contribute to the understanding and management of sarcoidosis in patients with concurrent chronic hepatitis B.
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Affiliation(s)
- Shigeaki Semba
- Department of Endoscopy, NHO Kure Medical Center, Chugoku Cancer Center, Hiroshima, Japan
| | - Yuji Teraoka
- Department of Gastroenterology, NHO Kure Medical Center, Chugoku Cancer Center, 3-1, Aoyama-cho, Kure, Hiroshima, 737-0023, Japan.
| | - Hiroki Kamada
- Department of Endoscopy, NHO Kure Medical Center, Chugoku Cancer Center, Hiroshima, Japan
| | - Naohiro Kato
- Department of Endoscopy, NHO Kure Medical Center, Chugoku Cancer Center, Hiroshima, Japan
| | - Takeshi Mizumoto
- Department of Endoscopy, NHO Kure Medical Center, Chugoku Cancer Center, Hiroshima, Japan
| | - Yuzuru Tamaru
- Department of Endoscopy, NHO Kure Medical Center, Chugoku Cancer Center, Hiroshima, Japan
| | - Tsuyoshi Hatakeyama
- Department of Endoscopy, NHO Kure Medical Center, Chugoku Cancer Center, Hiroshima, Japan
| | - Atsushi Yamaguchi
- Department of Gastroenterology, NHO Kure Medical Center, Chugoku Cancer Center, 3-1, Aoyama-cho, Kure, Hiroshima, 737-0023, Japan
| | - Hirotaka Kouno
- Department of Gastroenterology, NHO Kure Medical Center, Chugoku Cancer Center, 3-1, Aoyama-cho, Kure, Hiroshima, 737-0023, Japan
| | - Shigeto Yoshida
- Department of Endoscopy, NHO Kure Medical Center, Chugoku Cancer Center, Hiroshima, Japan
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3
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Sinnanaidu RP, Kumar VC, Shunmugam RH, Mahadeva S. The clinical management of hepatic sarcoidosis: A systematic review. JGH Open 2024; 8:e13076. [PMID: 38903487 PMCID: PMC11187478 DOI: 10.1002/jgh3.13076] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2023] [Revised: 04/11/2024] [Accepted: 04/18/2024] [Indexed: 06/22/2024]
Abstract
Background Hepatic sarcoidosis is an uncommon clinical condition in which clear recommendations are lacking in its treatment. We aimed to review systematically the literature on hepatic sarcoidosis treatment to guide clinicians. Methods Using MEDLINE, PubMed, CINAHL, Cochrane Library, and Google Scholar databases, we searched original articles on clinical studies reporting the outcome of adult hepatic sarcoidosis patients following treatment with various pharmacological agents. The primary end point was focused on assessing symptomatic relief and biochemical improvement posttreatment. Results Out of 614 retrieved references, 34 published studies were eligible, providing data for a total of 268 patients with hepatic sarcoidosis. First-line therapy with corticosteroids alone was reported in 187 patients, whilst ursodeoxycholic acid (UDCA) was used in 40 patients. Symptomatic and biochemical responses were reported among 113(60.4%) and 80(42.8%) cases of corticosteroids respectively, whereas UDCA showed a complete response in 23(57.5%) patients. Second-line therapy was used in steroid-refractory cases, with most cases being reported for azathioprine (n = 32) and methotrexate (n = 28). Notably, 15(46.9%) and 11(39.2%) patients showed both clinical and biochemical responses respectively. Biological therapy including anti-tumor necrosis factor (anti-TNF) was used as third line therapy in twelve cases with a 72.7% symptomatic and biochemical response rate each. Conclusion The quality of evidence for the treatment of hepatic sarcoidosis was poor. Nevertheless, it appears that corticosteroid or UDCA may be utilized as first-line therapy. For cases that are refractory to corticosteroids, steroid-sparing immunosuppressive agents and anti-TNF have shown some promising results, but further high-quality studies are required.
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Affiliation(s)
- Ram Prasad Sinnanaidu
- Gastroenterology Unit, Medical DepartmentUniversiti Malaya Medical CentreKuala LumpurMalaysia
| | | | - Ranita Hisham Shunmugam
- Department of Library & Information Science, Faculty of Arts & Social SciencesUniversiti MalayaKuala LumpurMalaysia
| | - Sanjiv Mahadeva
- Gastroenterology Unit, Medical DepartmentUniversiti Malaya Medical CentreKuala LumpurMalaysia
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4
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Kikuchi M, Koizumi A, Namisaki T, Asada S, Oyama M, Tomooka F, Fujimoto Y, Kitagawa K, Kawaratani H, Yoshiji H. Improvement of liver histology in hepatic sarcoidosis due to treatment with corticosteroids and ursodeoxycholic acid: a case report. Clin J Gastroenterol 2024; 17:327-333. [PMID: 38300407 DOI: 10.1007/s12328-023-01918-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/04/2023] [Accepted: 12/27/2023] [Indexed: 02/02/2024]
Abstract
We report the case of a 48-year-old male with a history of pulmonary and ocular sarcoidosis. Non-caseating granulomas, identified histologically, are the most characteristic manifestation of sarcoidosis. Hepatic sarcoidosis is difficult to diagnose using radiological imaging. In the patient reported in this study, ultrasound and contrast-enhanced computed tomography scans identified multiple intra-abdominal lymphadenopathies, with evidence of liver and splenic infiltrations. The first liver biopsy revealed non-caseating granulomatous hepatitis consistent with hepatic sarcoidosis. The patient was treated with ursodeoxycholic acid (UDCA), but his laboratory parameters did not improve. Prednisone was initiated at a dose of 30 mg daily and slowly tapered. At a dose of 12.5 mg daily, marked improvements in the fibrotic and sarcoid-like lesions were noted at the second biopsy. A third biopsy was performed, with the patient on a prednisone taper of 5 mg/day showed mild fibrous expansion in the portal tracts and mild parenchymal necro-inflammatory lesions. However, overall, fibrosis marker levels remained stable over the course of treatment. A fourth biopsy was performed after a 5-year course of 5 mg/day prednisone. This revealed minimal lobular inflammation without fibrosis. Thus, treatment of this patient with corticosteroids and UDCA resulted in marked improvements in his biochemical and histological parameters.
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Affiliation(s)
- Mayuko Kikuchi
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Aritoshi Koizumi
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Tadashi Namisaki
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan.
| | - Shouhei Asada
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Masafumi Oyama
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Fumimasa Tomooka
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Yuki Fujimoto
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Koh Kitagawa
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Hideto Kawaratani
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
| | - Hitoshi Yoshiji
- Department of Gastroenterology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara, Japan
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Abstract
In addition to being the primary target of infections such as viral hepatitis, the liver may also be affected by systemic disease. These include bacterial, mycotic, and viral infections, as well as autoimmune and infiltrative diseases. These conditions generally manifest as abnormal liver biochemistries, often with a cholestatic profile, and may present with additional signs/symptoms such as jaundice and fever. A high index of suspicion and familiarity with potential causal entities is necessary to guide appropriate testing, diagnosis, and treatment.
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Affiliation(s)
- Humberto C Gonzalez
- Division of Gastroenterology and Hepatology, Henry Ford Health, 2799 West Grand Boulevard, Detroit, MI 48202, USA; Wayne State University School of Medicine, 540 E Canfield St, Detroit, MI 48201, USA.
| | - Stuart C Gordon
- Division of Gastroenterology and Hepatology, Henry Ford Health, 2799 West Grand Boulevard, Detroit, MI 48202, USA; Wayne State University School of Medicine, 540 E Canfield St, Detroit, MI 48201, USA
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Hyzny EJ, Chan EG, Morrell M, Harano T, Sanchez PG. A review of liver dysfunction in the lung transplant patient. Clin Transplant 2021; 35:e14344. [PMID: 33960530 DOI: 10.1111/ctr.14344] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2020] [Revised: 04/09/2021] [Accepted: 04/26/2021] [Indexed: 12/11/2022]
Abstract
Liver dysfunction is an increasingly common finding in patients evaluated for lung transplantation. New or worsening dysfunction in the perioperative period, defined by presence of clinical ascites/encephalopathy, high model for end-stage liver disease (MELD) score, and/or independent diagnostic criteria, is associated with high short- and long-term mortality. Therefore, a thorough liver function assessment is necessary prior to listing for lung transplant. Unfortunately, identification and intraoperative monitoring remain the only options for prevention of disease progression with isolated lung transplantation. Combined lung and liver transplantation may provide an option for definitive long-term management in selecting patients with known liver disease at high risk for postoperative progression. However, experience with the combined operation is extremely limited and indications for combined lung and liver transplant remain unclear. Herein, we present a comprehensive literature review of patients with liver dysfunction undergoing lung transplantation with and without concurrent liver transplant in an effort to illuminate the risks, benefits, and clinical judgement surrounding decision to pursue combined lung-liver transplantation (CLLT). We also argue description of liver function is currently a weakness of the current lung allocation scoring system. Additional algorithms incorporating liver function may aid in risk stratification and decision to pursue combined transplantation.
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Affiliation(s)
- Eric J Hyzny
- Division of Thoracic Surgery, Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
| | - Ernest G Chan
- Division of Thoracic Surgery, Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
| | - Matthew Morrell
- Pulmonary, Allergy, and Critical Care Medicine Division, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
| | - Takashi Harano
- Division of Thoracic Surgery, Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
| | - Pablo G Sanchez
- Division of Thoracic Surgery, Department of Cardiothoracic Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
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Deliwala SS, Hussain M, Ponnapalli A, Khanal R, Goyal H, Abdalla A, Elbedawi MM. Sarcoidosis Masquerading as Long-Standing Cholestasis. Gastroenterology Res 2021; 14:112-115. [PMID: 34007353 PMCID: PMC8110232 DOI: 10.14740/gr1360] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/25/2021] [Accepted: 03/05/2021] [Indexed: 11/11/2022] Open
Abstract
Hepatic sarcoidosis is an exceedingly rare extrapulmonary manifestation of sarcoidosis, with the majority remaining stable for years without clinical clues, only displaying biochemical abnormalities. Amongst the literature, the timeline to cirrhosis has not been parsed out; hepatomegaly develops in 50% and cirrhosis in 33% of all hepatic sarcoidosis patients, making this an essential issue in this patient population. Interestingly, the risk for hepatocellular carcinoma remains high regardless of cirrhosis development. Corticosteroids and biologics remain the mainstay of therapy, although refractory cases may require deeper immunosuppression. Liver transplantation is seen in a handful of cases with promising results. We present an interesting case of cholestatic pattern livery injury in our outpatient setting that was eventually discovered to be hepatic sarcoidosis. Mild biochemical derangements or sole elevations in alkaline phosphatase are under-recognized, and patients often progress to cirrhosis and end-stage liver disease. This diagnostic miss has significant implications and represents an opportunity to treat liver disease with a reversible cause. Consensus guidelines recommend alkaline phosphatase screening in newly diagnosed cases of sarcoidosis.
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Affiliation(s)
- Smit S Deliwala
- Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, MI, USA
| | - Murtaza Hussain
- Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, MI, USA
| | - Anoosha Ponnapalli
- Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, MI, USA
| | - Rupesh Khanal
- Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, MI, USA
| | - Hemant Goyal
- Division of Gastroenterology, Department of Internal Medicine, The Wright Center for Graduate Medical Education, Scranton, PA, USA
| | - Adil Abdalla
- Division of Gastroenterology, Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, MI, USA
| | - Mamoon M Elbedawi
- Division of Gastroenterology, Department of Internal Medicine, Michigan State University at Hurley Medical Center, Flint, MI, USA
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8
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Brovko MY, Akulkina LA, Kalashnikov MV, Rozina TP, Nekrasova TP, Sholomova VI, Konovalov DV, Yanakayeva AS, Moiseev SV. [Sarcoidosis and primary biliary cholangitis in a patient with cholestasis]. TERAPEVT ARKH 2021; 93:193-198. [PMID: 36286636 DOI: 10.26442/00403660.2021.02.200620] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2021] [Accepted: 04/05/2021] [Indexed: 11/22/2022]
Abstract
Primary biliary cholangitis and sarcoidosis are both cholestatic liver diseases. Currently, there are no established specific criteria for distinguishing the diseases from each other; diagnosis is based on the anamnesis, as well as the results of physical, laboratory and instrumental examination. The case report presents a female patient with a rare combination of histologically verified liver sarcoidosis and primary biliary cholangitis. Despite the similar clinical manifestations, the approaches to the treatment of these diseases are completely different, that underlines the importance of the differential diagnosis to exclude combined liver damage.
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Affiliation(s)
- M Y Brovko
- Sechenov First Moscow State Medical University (Sechenov University)
| | - L A Akulkina
- Sechenov First Moscow State Medical University (Sechenov University)
| | - M V Kalashnikov
- Sechenov First Moscow State Medical University (Sechenov University)
| | - T P Rozina
- Sechenov First Moscow State Medical University (Sechenov University)
- Lomonosov Moscow State University
| | - T P Nekrasova
- Sechenov First Moscow State Medical University (Sechenov University)
| | - V I Sholomova
- Sechenov First Moscow State Medical University (Sechenov University)
- Lomonosov Moscow State University
| | - D V Konovalov
- Sechenov First Moscow State Medical University (Sechenov University)
| | - A S Yanakayeva
- Sechenov First Moscow State Medical University (Sechenov University)
| | - S V Moiseev
- Sechenov First Moscow State Medical University (Sechenov University)
- Lomonosov Moscow State University
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9
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Morris C, Drinkwater K, Varshney N. Hepatic Sarcoidosis Presenting as Hypercalcemia. Am J Med 2020; 133:e727-e728. [PMID: 32603790 DOI: 10.1016/j.amjmed.2020.05.031] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/06/2020] [Revised: 05/03/2020] [Accepted: 05/04/2020] [Indexed: 12/01/2022]
Affiliation(s)
- Craig Morris
- University of Arizona College of Medicine, Tucson, AZ
| | - Kathy Drinkwater
- Department of Internal Medicine, University of Arizona, Banner University Medical Center - South Campus, Tucson, AZ
| | - Neha Varshney
- Department of Pathology, University of Arizona, Banner University Medical Center - Tucson Campus, Tucson, AZ
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10
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De Mulder P, Maertens B, Hoorens A, Vonck A. Extrapulmonary sarcoidosis primarily presenting as cholestatic liver disease. BMJ Case Rep 2019; 12:12/12/e232618. [PMID: 31806633 DOI: 10.1136/bcr-2019-232618] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022] Open
Abstract
Sarcoidosis is a multisystem inflammatory disorder associated with non-caseating granulomas in affected organs, most commonly the lungs. Involvement of extrapulmonary organs is common, but lack of pulmonary involvement is rare and is called non-pulmonary sarcoidosis (NPS). Like pulmonary sarcoidosis, a definitive diagnostic test for NPS does not exist. Instead, the diagnosis of sarcoidosis requires the following elements: clinical and radiographic manifestations of sarcoidosis, histopathological detection of non-caseating granulomas and the exclusion of other diseases that may present similarly. Because of the experience with corticosteroids in pulmonary sarcoidosis, they are generally considered first-line therapy for NPS too. Ursodeoxycholic acid can be used to reduce cholestasis in NPS, but is inferior to corticosteroids in reducing inflammation. We hereby present a case that is particularly notable for its rare presentation of NPS as a granulomatous hepatitis with cholestatic liver function tests.
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Affiliation(s)
| | | | - Anne Hoorens
- Anatomopathology, Ghent University, Gent, Belgium
| | - An Vonck
- Nephrology, Algemeen Ziekenhuis Sint Blasius campus Dendermonde, Dendermonde, Belgium
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11
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Fomin VV, Brovko MY, Kalashnikov MV, Sholomova VI, Rozina TP, Akulkina LA, Pershina AE, Yanakayeva AS, Nekrasova TP. Hepatic involvement in sarcoidosis. TERAPEVT ARKH 2019; 91:8-16. [PMID: 31094470 DOI: 10.26442/00403660.2019.04.000179] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Liver is frequently involved in the pathological process. Wide range of clinical manifestations can be seen: from asymptomatic lesion with normal liver function tests to cirrhosis with portal hypertension. Biopsy plays the key role in diagnosis of the hepatic sarcoidosis. It is essential for morphological diagnosis to exclude other causes of granulomatous liver disease, most often - primary biliary cholangitis. Nowadays there are no standard treatment protocols for patients with hepatic sarcoidosis.
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Affiliation(s)
- V V Fomin
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Faculty of Medicine, Department of Internal Medicine No.1, Moscow, Russia
| | - M Yu Brovko
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), E.M. Tareev Clinic of Internal Diseases, Moscow, Russia
| | - M V Kalashnikov
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Medical and Preventive Faculty, Department of Internal, Occupational Diseases and Rheumatology, Moscow, Russia
| | - V I Sholomova
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), E.M. Tareev Clinic of Internal Diseases, Moscow, Russia.,I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Medical and Preventive Faculty, Department of Internal, Occupational Diseases and Rheumatology, Moscow, Russia
| | - T P Rozina
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), E.M. Tareev Clinic of Internal Diseases, Moscow, Russia.,M.V. Lomonosov Moscow State University, Faculty of Base Medicine, Department of Internal Medicine, Moscow, Russia
| | - L A Akulkina
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), E.M. Tareev Clinic of Internal Diseases, Moscow, Russia
| | - A E Pershina
- M.V. Lomonosov Moscow State University, Faculty of Base Medicine, Department of Internal Medicine, Moscow, Russia
| | - A Sh Yanakayeva
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), E.M. Tareev Clinic of Internal Diseases, Moscow, Russia
| | - T P Nekrasova
- I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), A.I. Strukov Department of Pathological Anatomy, Moscow, Russia
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12
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Abstract
Hepatic granulomas are a common finding in systemic sarcoidosis, but most patients remain asymptomatic. Elevated alkaline phosphatase is the most common sign of hepatic sarcoidosis (HS). Lacking a specific diagnostic test, the diagnosis of HS is one of exclusion. Therapy may be indicated in a minority of patients to control symptoms, but the effects of therapy in the natural history of HS are unknown.
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Affiliation(s)
- Manoj Kumar
- Division of Gastroenterology, University of South Alabama College of Medicine, UCOM 6000, 75 University Boulevard South, Mobile, AL 36688, USA
| | - Jorge L Herrera
- Division of Gastroenterology, University of South Alabama College of Medicine, UCOM 6000, 75 University Boulevard South, Mobile, AL 36688, USA.
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13
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Ibrahim AM, Bhandari B, Soriano PK, Quader Z, Gao JZ, Shuster D, Mamillapalli CK. Hepatic Involvement in Systemic Sarcoidosis. AMERICAN JOURNAL OF CASE REPORTS 2018; 19:1212-1215. [PMID: 30305603 PMCID: PMC6196583 DOI: 10.12659/ajcr.910600] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Patient: Female, 68 Final Diagnosis: Hepatic sarcoidosis Symptoms: Abdominal pain • nausea • weakness Medication: — Clinical Procedure: Liver biopsy Specialty: Gastroenterology and Hepatology
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Affiliation(s)
- Abdisamad M Ibrahim
- Department of Internal Medicine, Southern Illinois University School of Medicine, Springfield, IL, USA
| | - Bishal Bhandari
- Department of Internal Medicine, Southern Illinois University School of Medicine, Springfield, IL, USA
| | - Paolo K Soriano
- Department of Internal Medicine, Southern Illinois University School of Medicine, Springfield, IL, USA
| | - Zafar Quader
- Department of Gastroenterology and Hepatology, Southern Illinois University School of Medicine, Springfield, IL, USA
| | - John Z Gao
- Pathology Associates of Central Illinois, Memorial Medical Center, Springfield, IL, USA
| | - Dmitry Shuster
- Department of Gastroenterology and Hepatology, Springfield Clinic, Springfield, IL, USA
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14
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Masuda K, Takenaga S, Morikawa K, Kano A, Ojiri H. Hepatic sarcoidosis with atypical radiological manifestations: A case report. Radiol Case Rep 2018; 13:936-939. [PMID: 30105085 PMCID: PMC6077144 DOI: 10.1016/j.radcr.2018.06.013] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/25/2018] [Revised: 06/13/2018] [Accepted: 06/21/2018] [Indexed: 12/26/2022] Open
Abstract
Sarcoidosis is a multisystemic inflammatory disease of unknown origin characterized by the formation of noncaseating granulomas and accumulation of inflammatory cells. Sarcoidosis most commonly affects the lungs and lymphoid system. However, the liver can also be involved in 50%-65% of cases. On magnetic resonance imaging, sarcoidosis lesions usually present as hypointense lesions on all sequences. However, we present a rare case of nodular liver sarcoidosis presenting with T2 hyperintense lesions. In addition, while most cases of hepatic nodular sarcoidosis present with multiple small hepatic nodules, liver masses of our case are larger than usual. Moreover, this case suggested that when intact vascular structures penetrating liver nodular lesions are observed as in the current case, liver sarcoidosis can be included in a list of differential diagnosis.
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Affiliation(s)
- Koichi Masuda
- Department of Radiology, The Jikei University Katsushika Medical Center, 6-41-2, Aoto, Katsushika-ku, Tokyo 125-8506, Japan
| | - Shinsuke Takenaga
- Department of Radiology, The Jikei University Katsushika Medical Center, 6-41-2, Aoto, Katsushika-ku, Tokyo 125-8506, Japan
| | - Kazuhiko Morikawa
- Department of Radiology, The Jikei University Katsushika Medical Center, 6-41-2, Aoto, Katsushika-ku, Tokyo 125-8506, Japan
| | - Asami Kano
- Department of Radiology, The Jikei University Katsushika Medical Center, 6-41-2, Aoto, Katsushika-ku, Tokyo 125-8506, Japan
| | - Hiroya Ojiri
- Department of Radiology, The Jikei Medical University Hospital, 3-25-8, Nishishinbashi, Minato-ku, Tokyo 105-8461, Japan
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15
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Saito H, Ohmori M, Iwamuro M, Tanaka T, Wada N, Yasunaka T, Takaki A, Okada H. Hepatic and Gastric Involvement in a Case of Systemic Sarcoidosis Presenting with Rupture of Esophageal Varices. Intern Med 2017; 56:2583-2588. [PMID: 28883247 PMCID: PMC5658523 DOI: 10.2169/internalmedicine.8768-16] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/25/2016] [Accepted: 02/23/2017] [Indexed: 12/19/2022] Open
Abstract
A 46-year-old woman presented with massive hematemesis, caused by the rupture of esophageal varices. The laboratory investigations showed pancytopenia, and imaging tests revealed hepatosplenomegaly and ascites. A diagnosis of systemic sarcoidosis was made based on biopsies of the liver, stomach, lungs, heart, and skin. Although fat deposition was predominant, non-caseating granuloma and cirrhotic changes were found in the liver. Non-caseating granuloma was also identified in a biopsy specimen from minute depressions of the gastric folds. This case illustrates the rare involvement of the digestive system in a case of systemic sarcoidosis.
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Affiliation(s)
- Hiroaki Saito
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
- Saito Clinic, Japan
| | - Masayasu Ohmori
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
| | - Masaya Iwamuro
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
- Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
| | | | - Nozomu Wada
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
| | - Tetsuya Yasunaka
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
| | - Akinobu Takaki
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
| | - Hiroyuki Okada
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
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16
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Patedakis Litvinov BI, Pathak AP. Granulomatous hepatitis in a patient with Crohn's disease and cholestasis. BMJ Case Rep 2017; 2017:bcr-2017-220988. [PMID: 28882937 PMCID: PMC5589046 DOI: 10.1136/bcr-2017-220988] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/24/2017] [Indexed: 12/21/2022] Open
Abstract
We describe a case of a 23-year-old woman with a history of Crohn's disease (CD), who initially presented with sepsis-like symptoms, subsequently developed severe cholestasis and following extensive inpatient workup was found to have non-caseating granulomas on her liver biopsy. Infectious aetiologies were excluded and the patient was treated with oral corticosteroids, which ameliorated but did not completely reverse the cholestasis. We review the differential diagnosis of hepatic granulomas and discuss the potential difficulties in establishing their exact aetiology in patients with CD.
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Affiliation(s)
| | - Amit P Pathak
- Department of Internal Medicine, MedStar Washington Hospital Center, Washington, DC, USA
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17
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Al-Kofahi K, Korsten P, Ascoli C, Virupannavar S, Mirsaeidi M, Chang I, Qaqish N, Saketkoo LA, Baughman RP, Sweiss NJ. Management of extrapulmonary sarcoidosis: challenges and solutions. Ther Clin Risk Manag 2016; 12:1623-1634. [PMID: 27853374 PMCID: PMC5106225 DOI: 10.2147/tcrm.s74476] [Citation(s) in RCA: 49] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Background Sarcoidosis is a chronic multisystem disease of unknown etiology characterized by noncaseating granulomas that most often involves the lungs, but frequently has extrapulmonary manifestations, which might be difficult to treat in individual patients. Objective To review different disease manifestations, focusing on extrapulmonary organ systems, and to provide treatment options for refractory cases. Materials and methods We performed a literature search using Medline and Google Scholar for individual or combined keywords of “sarcoidosis, extrapulmonary, treatment, kidney, neurosarcoidosis, cardiovascular, gastrointestinal, transplantation, musculoskeletal, rheumatology, arthritis, and skin”. Peer-reviewed articles, including review articles, clinical trials, observational trials, and case reports that were published in English were included. References from retrieved articles were also manually searched for relevant articles. Results and conclusion Isolated involvement of a single organ or organ system is rare in sarcoidosis, and thus all patients must be thoroughly evaluated for additional disease manifestations. Cardiac sarcoidosis and neurosarcoidosis may be life-threatening. Clinicians need to assess patients comprehensively using clinical, laboratory, imaging, and histopathological data to recommend competently the best and least toxic treatment option for the individual patient.
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Affiliation(s)
- Khalid Al-Kofahi
- Department of Molecular Biosciences, University of Kansas, Lawrence, KS, USA
| | - Peter Korsten
- Department of Nephrology and Rheumatology, University Medical Center Göttingen, Göttingen, Germany
| | - Christian Ascoli
- Division of Pulmonary, Critical Care, Sleep and Allergy, University of Illinois at Chicago, Chicago, IL
| | | | - Mehdi Mirsaeidi
- Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Miami Miller School of Medicine, Miami, FL
| | - Ian Chang
- Department of Medicine, Michigan State University College of Osteopathic Medicine, East Lansing, MI
| | - Naim Qaqish
- Department of Gastroenterology, Hepatology and Nutrition, University of Buffalo, Buffalo, NY
| | - Lesley A Saketkoo
- New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, Louisiana State University Health Sciences Center, New Orleans, LA
| | - Robert P Baughman
- Department of Medicine, University Medical Center of Cincinnati, Cincinnati, OH, USA
| | - Nadera J Sweiss
- Division of Pulmonary, Critical Care, Sleep and Allergy, University of Illinois at Chicago, Chicago, IL; Division of Rheumatology, University of Illinois at Chicago, Chicago, IL
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18
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Palmucci S, Torrisi SE, Caltabiano DC, Puglisi S, Lentini V, Grassedonio E, Vindigni V, Reggio E, Giuliano R, Micali G, Caltabiano R, Andreula C, Foti PV, Ettorre GC, Walsh SL, Vancheri C. Clinical and radiological features of extra-pulmonary sarcoidosis: a pictorial essay. Insights Imaging 2016; 7:571-587. [PMID: 27222055 PMCID: PMC4956623 DOI: 10.1007/s13244-016-0495-4] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2015] [Revised: 04/07/2016] [Accepted: 04/22/2016] [Indexed: 12/19/2022] Open
Abstract
UNLABELLED The aim of this manuscript is to describe radiological findings of extra-pulmonary sarcoidosis. Sarcoidosis is an immune-mediated systemic disease of unknown origin, characterized by non-caseating epitheliod granulomas. Ninety percent of patients show granulomas located in the lungs or in the related lymph nodes. However, lesions can affect any organ. Typical imaging features of liver and spleen sarcoidosis include visceromegaly, with multiple nodules hypodense on CT images and hypointense on T2-weighted MRI acquisitions. Main clinical and radiological manifestations of renal sarcoidosis are nephrolithiasis, nephrocalcinosis, and acute interstitial nephritis. Brain sarcoidosis shows multiple or solitary parenchymal nodules on MRI that enhance with a ring-like appearance after gadolinium. In spinal cord localization, MRI demonstrates enlargement and hyperintensity of spinal cord, with hypointense lesions on T2-weighted images. Skeletal involvement is mostly located in small bone, showing many lytic lesions; less frequently, bone lesions have a sclerotic appearance. Ocular involvement includes uveitis, conjunctivitis, optical nerve disease, chorioretinis. Erythema nodosum and lupus pernio represent the most common cutaneous manifestations encountered. Sarcoidosis in various organs can be very insidious for radiologists, showing different imaging features, often non-specific. Awareness of these imaging features helps radiologists to obtain the correct diagnosis. TEACHING POINTS • Systemic sarcoidosis can exhibit abdominal, neural, skeletal, ocular, and cutaneous manifestations. • T2 signal intensity of hepatosplenic nodules may reflect the disease activity. • Heerfordt's syndrome includes facial nerve palsy, fever, parotid swelling, and uveitis. • In the vertebrae, osteolytic and/or diffuse sclerotic lesions can be found. • Erythema nodosum and lupus pernio represent the most common cutaneous manifestations.
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Affiliation(s)
- Stefano Palmucci
- Radiodiagnostic and Radiotherapy Unit, University Hospital "Policlinico-Vittorio Emanuele", Via Santa Sofia 78, 95123, Catania, Italy.
| | - Sebastiano Emanuele Torrisi
- Regional Centre for Interstitial and Rare Lung Diseases, Department of Clinical and Molecular Biomedicine, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123, Catania, Italy
| | - Daniele Carmelo Caltabiano
- Radiodiagnostic and Radiotherapy Unit, University Hospital "Policlinico-Vittorio Emanuele", Via Santa Sofia 78, 95123, Catania, Italy
| | - Silvia Puglisi
- Regional Centre for Interstitial and Rare Lung Diseases, Department of Clinical and Molecular Biomedicine, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123, Catania, Italy
| | - Viviana Lentini
- Unit of Diagnostic and Interventional Radiology, ARNAS Garibaldi, Catania, Italy
| | - Emanuele Grassedonio
- Section of Radiological Sciences, DIBIMEF, University Hospital "Paolo Giaccone" University of Palermo, Palermo, Italy
| | - Virginia Vindigni
- Regional Centre for Interstitial and Rare Lung Diseases, Department of Clinical and Molecular Biomedicine, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123, Catania, Italy
| | - Ester Reggio
- Department of G. F. Ingrassia, Section of Neurosciences, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123, Catania, Italy
| | - Riccardo Giuliano
- Regional Centre for Interstitial and Rare Lung Diseases, Department of Clinical and Molecular Biomedicine, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123, Catania, Italy
| | | | - Rosario Caltabiano
- Department of G.F. Ingrassia, Institute of Pathology, University Hospital Policlinico-Vittorio Emanuele, Catania, Italy
| | - Cosma Andreula
- Neuroradiology and Radiology, Anthea Hospital Bari, Gruppo Villa Maria, Puglia, Italy
| | - Pietro Valerio Foti
- Radiodiagnostic and Radiotherapy Unit, University Hospital "Policlinico-Vittorio Emanuele", Via Santa Sofia 78, 95123, Catania, Italy
| | - Giovanni Carlo Ettorre
- Radiodiagnostic and Radiotherapy Unit, University Hospital "Policlinico-Vittorio Emanuele", Via Santa Sofia 78, 95123, Catania, Italy
| | - Simon Lf Walsh
- Department of Radiology, Kings College Hospital Foundation Trust, Denmark Hill, London, UK
| | - Carlo Vancheri
- Regional Centre for Interstitial and Rare Lung Diseases, Department of Clinical and Molecular Biomedicine, University Hospital Policlinico-Vittorio Emanuele, Via Santa Sofia 78, 95123, Catania, Italy
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19
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Ennaifer R, Ayadi S, Romdhane H, Cheikh M, Nejma HB, Bougassas W, Hadj NB. Hepatic sarcoidosis: a case series. Pan Afr Med J 2016; 24:209. [PMID: 27795804 PMCID: PMC5072846 DOI: 10.11604/pamj.2016.24.209.7980] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2015] [Accepted: 09/30/2015] [Indexed: 12/30/2022] Open
Abstract
Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven.
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Affiliation(s)
- Rym Ennaifer
- Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia
| | - Shema Ayadi
- Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia
| | - Hayfa Romdhane
- Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia
| | - Myriam Cheikh
- Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia
| | - Houda Ben Nejma
- Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia
| | - Wassila Bougassas
- Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia
| | - Najet Bel Hadj
- Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia
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20
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Sghier IA, Billah NM. [Budd-Chiari syndrome: a rare complication of hepatic sarcoidosis (about one case)]. Pan Afr Med J 2016; 23:4. [PMID: 27200114 PMCID: PMC4856515 DOI: 10.11604/pamj.2016.23.4.8564] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2015] [Accepted: 12/21/2015] [Indexed: 12/02/2022] Open
Abstract
L'atteinte hépatique au cours de la sarcoïdose est une localisation fréquente, habituellement asymptomatique. La cholestase anictérique et l'hypertension portale représentent ses principales complications. Le syndrome de Budd-Chiari est une complication peu connue qui demeure exceptionnelle. Nous rapportons un nouveau cas de syndrome de Budd-Chiari compliquant une sarcoïdose hépatique chez une jeune femme de 45 ans.
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21
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Ennaifer R, Bacha D, Romdhane H, Cheikh M, Nejma HB, BelHadj N. Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis. Clin Pract 2015; 5:768. [PMID: 26900444 PMCID: PMC4736049 DOI: 10.4081/cp.2015.768] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2015] [Revised: 04/28/2015] [Accepted: 06/23/2015] [Indexed: 12/21/2022] Open
Abstract
Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties.
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Affiliation(s)
- Rym Ennaifer
- Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis
- Faculty of Medicine, University of Tunis El Manar, Tunis
| | - Dhouha Bacha
- Faculty of Medicine, University of Tunis El Manar, Tunis
- Department of Pathology, Mongi Slim Hospital, Tunis, Tunisia
| | - Hayfa Romdhane
- Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis
- Faculty of Medicine, University of Tunis El Manar, Tunis
| | - Myriam Cheikh
- Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis
- Faculty of Medicine, University of Tunis El Manar, Tunis
| | - Houda Ben Nejma
- Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis
- Faculty of Medicine, University of Tunis El Manar, Tunis
| | - Najet BelHadj
- Department of Hepato-Gastro-Enterology, Mongi Slim Hospital, Tunis
- Faculty of Medicine, University of Tunis El Manar, Tunis
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22
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Modaresi Esfeh J, Culver D, Plesec T, John B. Clinical presentation and protocol for management of hepatic sarcoidosis. Expert Rev Gastroenterol Hepatol 2015; 9:349-358. [PMID: 25473783 DOI: 10.1586/17474124.2015.958468] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
The liver is one of the most commonly involved extrapulmonary sites in sarcoidosis. Hepatic sarcoidosis has a broad range of presentations from scattered, asymptomatic noncaseating granulomas with normal liver enzymes, which are very common in patients with known pulmonary sarcoidosis, to portal hypertension and cirrhosis, which are relatively uncommon. Diagnosis is based on a combination of clinical, laboratory and histological manifestations. The authors' protocol for management of patients with suspected sarcoidosis of the liver without focal lesions includes a transjugular liver biopsy with portal pressure measurements to confirm the diagnosis, rule out coexisting liver diseases and to identify select patients with fibrosis or portal hypertension for consideration of immunosuppression. Steroids and azathioprine are the preferred agents and methotrexate is not recommended.
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23
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Stitt RS, Greenwood R, Laczek J. A surprising cause of acute right upper quadrant pain. BMJ Case Rep 2014; 2014:bcr-2013-201943. [PMID: 25103316 DOI: 10.1136/bcr-2013-201943] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
A 42 year-old African-American woman was admitted for severe acute right upper quadrant pain. Her liver function tests showed a cholestatic pattern of hepatitis. She had no known history of liver disease or sarcoidosis. Imaging of her liver and biliary tree did not reveal any apparent cause for her right upper quadrant pain. A liver biopsy was performed which showed granulomatous disease. This prompted a CT chest that showed mediastinal lymphadenopathy. Biopsy of the mediastinal lymphnode revealed non-caseating granulomas. Despite having no pulmonary symptoms or history of pulmonary sarcoidosis, she was diagnosed with systemic pulmonary sarcoidosis. She was treated with corticosteroids and had complete resolution of symptoms over the next several weeks.
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Affiliation(s)
- Rodger Scott Stitt
- Department of Medicine, Tripler Army Medical Center, Honolulu, Hawaii, USA
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24
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Savvidou S, Gagalis A, Iosifidou M, Kalambakas A. A 60-year-old man with fever, cholestasis and granulomatous hepatitis. Clin Res Hepatol Gastroenterol 2014; 38:252-5. [PMID: 24525009 DOI: 10.1016/j.clinre.2014.01.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2013] [Revised: 12/16/2013] [Accepted: 01/07/2014] [Indexed: 02/04/2023]
Affiliation(s)
- Savvoula Savvidou
- 1st Department of Internal Medicine, "Papageorgiou" General Hospital of Thessaloniki, Ring Road N. Efkarpia-Thessaloniki, 56429 Thessaloniki, Greece.
| | - Asterios Gagalis
- 1st Department of Internal Medicine, "Papageorgiou" General Hospital of Thessaloniki, Ring Road N. Efkarpia-Thessaloniki, 56429 Thessaloniki, Greece
| | - Maria Iosifidou
- 1st Department of Internal Medicine, "Papageorgiou" General Hospital of Thessaloniki, Ring Road N. Efkarpia-Thessaloniki, 56429 Thessaloniki, Greece
| | - Athanasios Kalambakas
- 1st Department of Internal Medicine, "Papageorgiou" General Hospital of Thessaloniki, Ring Road N. Efkarpia-Thessaloniki, 56429 Thessaloniki, Greece
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25
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Kurosaki F, Bando M, Nakayama M, Mato N, Yamasawa H, Higashizawa T, Tanaka A, Sugiyama Y. A patient with sarcoidosis who developed heterochronic involvements in different organs from initial organs during 7 years. Respir Investig 2014; 52:71-4. [PMID: 24388374 DOI: 10.1016/j.resinv.2013.05.003] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2013] [Revised: 05/14/2013] [Accepted: 05/17/2013] [Indexed: 10/26/2022]
Abstract
A 59-year-old woman, who was given a diagnosis of sarcoidosis by supraclavicular lymph node biopsy 5 years previously, was admitted for further examination following abnormal radiologic findings. Nodular pulmonary and abdominal lesions were observed by computed tomography, and liver biopsy was performed and showed epithelioid cell granulomas. She was asymptomatic and was followed up with no therapy. At 1 year follow-up, the pulmonary and abdominal lesions had nearly complete resolution. Nodular pulmonary and abdominal lesions in patients with sarcoidosis can mimic metastatic disease, lymphoma, and infection, and can reappear during disease activity. Therefore, differential diagnosis and continual follow-up are important.
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Affiliation(s)
- Fumio Kurosaki
- Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
| | - Masashi Bando
- Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
| | - Masayuki Nakayama
- Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
| | - Naoko Mato
- Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
| | - Hideaki Yamasawa
- Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
| | - Toshihiko Higashizawa
- Division of Gastroenterology and Hepatology, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
| | - Akira Tanaka
- Division of Pathology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
| | - Yukihiko Sugiyama
- Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke city, Tochigi, Japan.
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26
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Tadros M, Forouhar F, Wu GY. Hepatic Sarcoidosis. J Clin Transl Hepatol 2013; 1:87-93. [PMID: 26357609 PMCID: PMC4521279 DOI: 10.14218/jcth.2013.00016] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2013] [Revised: 10/09/2013] [Accepted: 10/09/2013] [Indexed: 12/20/2022] Open
Abstract
Sarcoidosis is a multisystem disease characterized by the presence of non-caseating granulomas in affected organs. Pulmonary involvement is the most common site of disease activity. However, hepatic involvement is also common in sarcoidosis, occurring in up to 70% of patients. Most patients with liver involvement are asymptomatic. Therefore, the majority of cases are discovered incidentally, frequently by the finding of elevated liver enzymes. Pain in the right upper quadrant of the abdomen, fatigue, pruritus, and jaundice may be associated with liver involvement. Portal hypertension and cirrhosis are complications linked to long-standing hepatic sarcoidosis. Liver biopsy is usually required to confirm the diagnosis. It is important to differentiate hepatic sarcoidosis from other autoimmune and granulomatous liver diseases. Not all cases of hepatic sarcoidosis require treatment. For symptomatic patients, the first line treatment includes corticosteroids or ursodeoxycholic acid. Various immunosuppressant agents can be used as second line agents. Rarely, severe cases require liver transplantation.
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Affiliation(s)
- Micheal Tadros
- Department of Medicine, Division of Gastroenterology-Hepatology, University of Connecticut Health Center, Farmington, CT, USA
| | - Faripour Forouhar
- Department of Anatomic Pathology, University of Connecticut Health Center, Farmington, CT, USA
| | - George Y. Wu
- Department of Medicine, Division of Gastroenterology-Hepatology, University of Connecticut Health Center, Farmington, CT, USA
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27
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Ferreira A, Ramalho M, de Campos RO, Heredia V, Roque A, Vaidean G, Semelka RC. Hepatic sarcoidosis: MR appearances in patients with chronic liver disease. Magn Reson Imaging 2013; 31:432-8. [DOI: 10.1016/j.mri.2012.08.005] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2012] [Revised: 08/17/2012] [Accepted: 08/30/2012] [Indexed: 10/27/2022]
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28
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Aravinthan A, Gelson W, Limbu A, Brais R, Richardson P. Hepatic sarcoidosis complicating treatment-naive viral hepatitis. World J Hepatol 2012; 4:402-5. [PMID: 23355920 PMCID: PMC3554806 DOI: 10.4254/wjh.v4.i12.402] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/15/2011] [Revised: 03/05/2012] [Accepted: 11/14/2012] [Indexed: 02/06/2023] Open
Abstract
Hepatic sarcoidosis is usually asymptomatic but rarely leads to adverse liver-related outcome. Co-existence of viral hepatitis and hepatic sarcoidosis is a rare, but recognised phenomenon. Obtaining a balance between immune suppression and anti-viral therapy may be problematic. Immunosuppression in the presence of viral hepatitis can lead to rapid deterioration of liver disease. Similarly, anti-viral therapy may exacerbate granulomatous hepatitis. Here we present two cases of viral hepatitis co-existing with sarcoidosis that illustrate successful management strategies. In one, hepatitis B replication was suppressed with oral anti-viral therapy before commencing prednisolone. In the second, remission of hepatic sarcoidosis was achieved with prednisolone, before treating hepatitis C and obtaining a sustained virological response with pegylated interferon and ribavirin therapy.
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Affiliation(s)
- Aloysious Aravinthan
- Aloysious Aravinthan, William Gelson, Anita Limbu, Department of Hepatology, Cambridge University Hospitals NHS Trust, Cambridge CB2 0QQ, United Kingdom
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Abstract
PURPOSE OF REVIEW Surveillance of hepatic involvement in sarcoidosis has not been standardized. Therefore, management of hepatic involvement is a clinical challenge. This review analyses published data on the pharmacological treatment of hepatic sarcoidosis. RECENT FINDINGS Only 5-30% of patients with hepatic sarcoidosis display symptoms. Occasionally, it has a rapid progressive course with serious complications, stressing an appropriate and carefully timed therapeutic approach. Because symptomatic hepatic sarcoidosis is uncommon, therapeutic studies are scarce. Answers to the questions when to initiate which treatment are lacking. Case reports describe beneficial effects of prednisone and the augmentation of cytotoxic and anti-tumor necrotic factor-α (TNF-α) therapy. However, because of small sample sizes, no meaningful conclusions could be drawn. In symptomatic hepatic sarcoidosis patients, it is recommended to start to treat the sarcoidosis with prednisone, preceded by ursodeoxycholic acid when signs of cholestasis are present. In refractory cases or when prednisone weaning is impossible, cytotoxic drugs or anti-TNF-α therapy should be considered. SUMMARY This review illustrates the importance of an appropriate therapeutic approach of sarcoidosis patients with hepatic involvement. It emphasizes the need for future studies to evaluate treatment options to avoid disease progression and hepatic complications.
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Elloumi H, Marzouk S, Tahri N, Bahloul Z, Azouz M. Sarcoïdose et atteinte hépatique : étude de 25 cas. Rev Med Interne 2012; 33:607-14. [DOI: 10.1016/j.revmed.2012.08.007] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2012] [Revised: 05/31/2012] [Accepted: 08/07/2012] [Indexed: 02/01/2023]
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Brjalin V, Salupere R, Tefanova V, Prikk K, Lapidus N, Jõeste E. Sarcoidosis and chronic hepatitis C: A case report. World J Gastroenterol 2012; 18:5816-20. [PMID: 23155326 PMCID: PMC3484354 DOI: 10.3748/wjg.v18.i40.5816] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/17/2012] [Revised: 06/19/2012] [Accepted: 08/14/2012] [Indexed: 02/06/2023] Open
Abstract
Several case reports deal with the relationship between hepatitis C virus (HCV) infection and pulmonary or hepatic sarcoidosis. Most publications describe interferon α-induced sarcoidosis. However, HCV infection per se is also suggested to cause sarcoidosis. The present case report describes a case of biopsy-verified lung and liver sarcoidosis and HCV infection, and the outcome of antiviral therapy. In March 2009, a 25-year-old man presented with moderately elevated liver enzymes without any clinical symptoms. The patient was positive for HCV antibodies and HCV RNA of genotype 1b. Four months later the patient became dyspnoic and pulmonary sarcoidosis was diagnosed by lung biopsy and radiography. A short course of corticosteroid treatment relieved symptoms. Three months later, liver biopsy showed noncaseating granulomas consisting of epithelioid histiocytes and giant cells with a small amount of peripheral lymphocyte infiltration, without any signs of fibrosis. Chronic HCV infection with coexistence of pulmonary and hepatic sarcoidosis was diagnosed. Antiviral therapy with peginterferon α and ribavirin at standard doses was started, which lasted 48 wk, and sustained viral response was achieved. A second liver biopsy showed disappearance of granulomas and chest radiography revealed normalization of mediastinal and perihilar glands. The hypothesis that HCV infection per se may have triggered systemic sarcoidosis was proposed. Successful treatment of HCV infection led to continuous remission of pulmonary and hepatic sarcoidosis. Further studies are required to understand the relationship between systemic sarcoidosis and HCV infection.
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32
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Abstract
Hepatic granulomata are not infrequently encountered in liver biopsy and often are associated with systemic disease. The clinical presentation varies with the particular systemic process. From a biochemical standpoint, the most common abnormalities are elevated serum alkaline phosphatase and γ-glutamyltransferase. The observation of granulomata in a liver biopsy specimen warrants workup to identify a possible cause. Clues may be obtained in the medical history, on physical examination, or with specialized blood testing or radiologic studies. Treatment involves therapy of the underlying cause of the disease associated with the development of the granulomatous hepatitis.
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Affiliation(s)
- Steven L Flamm
- Division of Hepatology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
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33
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Abstract
Cholestasis develops either from a defect in bile synthesis, impairment in bile secretion, or obstruction to bile flow, and is characterized by an elevated serum alkaline phosphatase and gamma-glutamyltransferase disproportionate to elevation of aminotransferase enzymes. Key elements to the diagnostic workup include visualization of the biliary tree by cholangiography and evaluation of liver histology. The hope is that recent advances in understanding the genetic factors and immune mechanisms involved in the pathogenesis of cholestasis will lead to newer therapeutic interventions in the treatment of these diseases.
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Affiliation(s)
- Asma Siddique
- Department of Gastroenterology, Center for Liver Disease, Digestive Disease Institute, Seattle, WA 98111, USA
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34
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Abstract
BACKGROUND Sarcoidosis is a multisystemic inflammatory granulomatous disease. The prevalence of hepatic involvement is not clear. AIM The aim of this study was to establish the presence and severity of the liver-test abnormalities in sarcoidosis. METHODS Retrospectively, patients with confirmed sarcoidosis (n=837) presented with the liver-test abnormalities [alkaline phosphatase, γ-glutamyl transaminase, alanine aminotransferase or aspartate aminotransferase >1.5 times the upper limit of normal (ULN)] who were classified according to severity into mild (zero liver tests ≥3×ULN), moderate (one or two liver tests ≥3×ULN) and severe (three or four liver tests ≥3×ULN) were evaluated. Moreover, the relationship between severity of liver tests and histology was examined. RESULTS Liver-test abnormalities were found in 204 of 837 patients with chronic sarcoidosis (24.4%), among which 127 (15.2%) were suspected of having hepatic sarcoidosis (79 of 127 males, 111 Caucasian, eight African-American). In 22 of 127 patients (17.3%), a liver biopsy was obtained; 21 were compatible with hepatic sarcoidosis. In these 21 patients, severity of liver-test abnormalities was significantly associated with extensiveness of granulomatous inflammation (ρ=0.58, P=0.006) and degree of fibrosis (ρ=0.64, P=0.002). These results remained in the multiple regression analysis when controlled for treatment status, sex, genetics, ethnicity and age. CONCLUSION Liver-test abnormalities were present in 24% of the studied patients; in 15% highly because of hepatic involvement of sarcoidosis. Moderate and severe liver-test abnormalities seemed to be associated with more advanced histopathological disease. Therefore, in the management of sarcoidosis, for patients with moderate or severe liver-test abnormalities a liver biopsy is recommended.
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Vanatta JM, Modanlou KA, Dean AG, Nezakatgoo N, Campos L, Nair S, Eason JD. Outcomes of orthotopic liver transplantation for hepatic sarcoidosis: an analysis of the United Network for Organ Sharing/Organ Procurement and Transplantation Network data files for a comparative study with cholestatic liver diseases. Liver Transpl 2011; 17:1027-34. [PMID: 21594966 DOI: 10.1002/lt.22339] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Hepatic sarcoidosis is a rare indication for liver transplantation. Using the United Network for Organ Sharing (UNOS)/Organ Procurement and Transplantation Network (OPTN) database, we evaluated patient and graft survival after orthotopic liver transplantation for sarcoidosis between October 1987 and December 2007. We assessed the potential prognostic value of multiple demographic and clinical variables, and we also compared these patients to a case-matched group of patients with primary sclerosing cholangitis (PSC) or primary biliary cirrhosis (PBC). The 1- and 5-year survival rates for the sarcoidosis group were 78% and 61%, respectively, and these rates were significantly worse than the rates for the PSC/PBC group (P = 0.001). Disease recurrence in the liver is a rare cause of graft loss or patient death. Three deaths occurred in the sarcoidosis group because of recurrent hepatic sarcoidosis, and 1 death was a result of cardiac sarcoidosis. A univariate analysis identified an increasing donor risk index as a significant negative factor for outcomes for the sarcoidosis group [hazard ratio (HR) = 2.06, confidence interval (CI) = 1.04-4.06, P = 0.037], but this finding was not found in a multivariate analysis, in which no independent predictors were found to have a significant impact. A case-matched univariate analysis demonstrated that sarcoidosis and morbid obesity were significant negative factors for outcomes, and in a multivariate analysis, sarcoidosis continued to predict worse outcomes (HR = 2.39, CI = 1.21-4.73, P = 0.012). In conclusion, an analysis of the UNOS/OPTN database indicates that the patient and allograft survival rates for hepatic sarcoidosis are satisfactory, but they are worse in comparison with the rates for other cholestatic liver diseases.
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Affiliation(s)
- Jason M Vanatta
- Division of Transplantation, University of Tennessee, Methodist University Hospital Transplant Institute, Memphis, TN 38104, USA.
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36
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Mosea H, Gotto J, Khan Z. Diagnostic and therapeutic challenges of hepatic sarcoidosis. BMJ Case Rep 2011; 2011:bcr.04.2011.4069. [PMID: 22689553 DOI: 10.1136/bcr.04.2011.4069] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
The authors report three cases of hepatic sarcoidosis complicated by portal hypertension in Caucasian patients. The cases varied in their presentations from normal liver function to symptomatic cholestasis and established liver cirrhosis. In one particular case the authors discovered hepatic sarcoid after a diagnostic gastroscopy for upper gastrointestinal (GI) bleeding which showed changes of portal hypertension. In all of these cases the authors gave our patients a prolonged course of immunosuppressive treatment with over all good long-term results. The authors hope that our work will provide a modern approach into treating this disease.
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Affiliation(s)
- Haider Mosea
- Gastroenterology Department, Cheltenham General Hospital, Cheltenham, Gloucestershire, UK.
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Lagana SM, Moreira RK, Lefkowitch JH. Hepatic granulomas: pathogenesis and differential diagnosis. Clin Liver Dis 2010; 14:605-17. [PMID: 21055685 DOI: 10.1016/j.cld.2010.07.005] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Granulomatous liver disease constitutes a category of hepatic disorders and is at present diagnosed in approximately 4% of liver biopsies. Hepatic granulomas develop through the interactions of T lymphocytes and macrophages, with the integral involvement of T-helper (T(H)) 1 or T(H)2 pathways or both, depending on the specific granulomatous disease. Hepatic granulomas may be manifested clinically by elevated levels of serum alkaline phosphatase and g-glutamyltransferase enzymes, damage to specific structures (eg, intrahepatic bile ducts in primary biliary cirrhosis), or infrequently, progressive liver disease with portal hypertension and cirrhosis (eg, sarcoidosis). Systemic immunologic disorders, infectious diseases, drug hepatotoxicity, and reaction to neoplastic disease are the major causative factors responsible for granulomas in the liver. These causes and recent epidemiologic trends are covered in this discussion.
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Affiliation(s)
- Stephen M Lagana
- Department of Pathology and Cell Biology, Columbia University Medical Center, New York, NY 10032, USA.
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Delfosse V, de Leval L, De Roover A, Delwaide J, Honoré P, Boniver J, Detry O. Budd-Chiari syndrome complicating hepatic sarcoidosis: definitive treatment by liver transplantation: a case report. Transplant Proc 2010; 41:3432-4. [PMID: 19857764 DOI: 10.1016/j.transproceed.2009.09.021] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022]
Abstract
Sarcoidotic involvement of the liver is frequent, albeit uncommonly symptomatic. Severe complications are rare, but may seldom require liver transplantation. Budd-Chiari syndrome has been described in a few patients with hepatic sarcoidosis. Herein we have reported the case of a young woman suffering from hepatic sarcoidosis who developed severe cholestasis and chronic Budd-Chiari syndrome. She successfully underwent orthotopic liver transplantation (OLT) and is asymptomatic with normal liver function at 3 years follow-up. Histopathological assessment of the liver explant demonstrated a florid granulomatous process, with involvement of the large intrahepatic veins, providing an anatomical basis for the vascular flow disturbances. This case adds further evidence that liver transplantation may be the curative treatment for complicated sarcoidotic liver disease.
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Affiliation(s)
- V Delfosse
- Department of Pathology, CHU de Liège, University of Liège, B4000 Liège, Belgium
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Martin-Blondel G, Camara B, Selves J, Robic MA, Thebault S, Bonnet D, Alric L. Étiologies et évolution des hépatites granulomateuses : étude rétrospective de 21 cas consécutifs. Rev Med Interne 2010; 31:97-106. [DOI: 10.1016/j.revmed.2009.10.430] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2009] [Revised: 09/19/2009] [Accepted: 10/12/2009] [Indexed: 11/24/2022]
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40
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Ketari Jamoussi S, Mâamouri N, Ben Dhaou B, Baili L, Boussema F, Kochbati S, Cherif O, Ben Mami N, Rokbani L. L’atteinte hépatique au cours de la sarcoïdose : à propos de sept cas. Rev Med Interne 2010; 31:12-6. [DOI: 10.1016/j.revmed.2009.04.006] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/09/2008] [Revised: 04/07/2009] [Accepted: 04/28/2009] [Indexed: 11/29/2022]
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41
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Maàmouri N, Ketari S, Habessi H, Nouira K, Ben Dhaou B, Boussema F, Cherif O, Ben Mami N, Rokbani L. [Budd-Chiari syndrome in sarcoidosis]. ACTA ACUST UNITED AC 2009; 33:147-8. [PMID: 19157737 DOI: 10.1016/j.gcb.2008.11.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2008] [Revised: 09/27/2008] [Accepted: 11/03/2008] [Indexed: 10/21/2022]
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42
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Abstract
Sarcoid affecting the skin, eye, or liver can be symptomatic of or cause significant morbidity. When disease is sever, alternative therapies may be needed.
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Affiliation(s)
- Anthony S Rose
- Division of Pulmonary and Critical Care Medicine, Indiana University, Richard L. Roudebush VA Medical Center, 1481 W. 10th Street, Indianapolis, IN 46202, USA
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Kishor S, Turner ML, Borg BB, Kleiner DE, Cowen EW. Cutaneous sarcoidosis and primary biliary cirrhosis: A chance association or related diseases? J Am Acad Dermatol 2008; 58:326-35. [PMID: 18222332 DOI: 10.1016/j.jaad.2007.07.031] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2007] [Revised: 07/02/2007] [Accepted: 07/24/2007] [Indexed: 12/26/2022]
Abstract
Sarcoidosis and primary biliary cirrhosis (PBC) are thought to be two distinct disorders of unknown origin. However, both are characterized by hepatic granuloma formation and may also manifest cutaneous granulomatous inflammation. In this report, we describe two cases of cutaneous sarcoidosis occurring in the setting of PBC and review 7 additional cases from the literature of granulomatous skin disease associated with PBC. Although the pathogenesis of both sarcoidosis and PBC remains elusive, the simultaneous occurrence of these uncommon diseases suggests a common pathway may contribute to granuloma formation in both disorders.
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Affiliation(s)
- Sowmya Kishor
- Department of Dermatology, Eastern Virginia Medical School, Norfolk, Virginia, USA
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44
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Abstract
The liver has a double blood supply and plays a central role in the metabolism of proteins, carbohydrates, and many medications. In addition, it has a role in the induction of immune tolerance and may also be a target for immune-mediated damage. For these reasons, the liver may be involved in many systemic diseases. In this review, we discuss the involvement of the liver in granulomatous, rheumatologic, malignant, and circulatory diseases. An understanding of the wide spectrum of liver involvement in systemic diseases will aid in both diagnosis and treatment of patients with a wide range of medical conditions.
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Affiliation(s)
- Stephen Malnick
- Department of Internal Medicine, Institute of Gastroenterology , Kaplan Medical Center, Rehovot, Israel.
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45
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Bourdillon L, Lanier-Gachon E, Stankovic K, Bancel B, Lapras V, Broussolle C, Sève P. Löfgren Syndrome and Peritoneal Involvement by Sarcoidosis. Chest 2007; 132:310-2. [PMID: 17625092 DOI: 10.1378/chest.06-2996] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
Abstract
OBJECTIVE An unusual case of a patient with Löfgren syndrome peritoneal involvement by sarcoidosis. PATIENT AND METHODS A 36-year-old woman presented with Löfgren syndrome and an increase in liver enzyme levels. An abdominal CT scan showed multiple nodules on the peritoneum mimicking peritoneal carcinomatosis. Laparoscopy was conducted with biopsy of the peritoneal nodules. RESULTS Biopsy specimens from the peritoneum, liver, and bronchi showed noncaseating granulomas, and the search for tuberculosis was negative. Clinical and biological features resolved within 6 months, without therapy with steroids, while a thoracic CT scan as well as an abdominal CT scan showed no change. CONCLUSION To our knowledge, this is the first reported case of peritoneal sarcoidosis associated with Löfgren syndrome. A longer follow-up will, however, be required to assess the chronicity of the disease.
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Affiliation(s)
- Laure Bourdillon
- Department of Internal Medicine, Hôtel Dieu, 69288 Lyon Cedex 02, France
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Abstract
Sarcoidosis is a systemic inflammatory disorder of unknown etiology. Although any organ may be involved, the lungs are most frequently affected. The clinical course of the disease is highly variable, with up to two-thirds of untreated patients experiencing spontaneous remission within 12-24 months of onset of symptoms. When therapy is required, corticosteroids are considered standard, but studies demonstrating their ability to modify the long-term outcome in this disease are lacking. Often, the myriad of adverse side effects of corticosteroids necessitate the addition of immunosuppressants, cytotoxic agents or biologic therapies to maintain disease remission. Unfortunately, optimal therapeutic regimens have not been described. Patients who do not respond to therapy often experience progressive fibrotic changes and end-organ damage, which ultimately may result in significant morbidity or death. Agents commonly used to treat patients with sarcoidosis and emerging therapeutic options are discussed.
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Affiliation(s)
- Eric S White
- University of Michigan Medical Center, Division of Pulmonary and Critical Medicine, Department of Internal Medicine, 6301 MSRB III/0642, 1150 W. Medical Center Drive, Ann Arbor, MI 48109-0642, USA.
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