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Kitamura W, Kuratsune M, Iseki A, Kuyama S. ANCA-associated vasculitis with isolated splenomegaly as the initial organ presentation. Rheumatol Adv Pract 2024; 8:rkae045. [PMID: 38618140 PMCID: PMC11014779 DOI: 10.1093/rap/rkae045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/24/2024] [Indexed: 04/16/2024] Open
Affiliation(s)
- Wataru Kitamura
- Department of Hematology, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Japan
- Department of Hematology, Oncology and Respiratory Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan
| | - Masatoshi Kuratsune
- Department of Nephrology, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Japan
| | - Akiko Iseki
- Department of Pathology, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Japan
| | - Shoichi Kuyama
- Department of Respiratory Medicine, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Japan
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2
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Correia J, Gomes C, Ponte A, João D, Freitas T. Overt Bleeding from Small Bowel Ulcers due to Microscopic Polyangiitis. Middle East J Dig Dis 2022; 14:488-490. [PMID: 37547503 PMCID: PMC10404103 DOI: 10.34172/mejdd.2022.312] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/02/2022] [Accepted: 09/04/2022] [Indexed: 08/08/2023] Open
Affiliation(s)
- João Correia
- Gastroenterology Department, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
| | - Catarina Gomes
- Gastroenterology Department, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
| | - Ana Ponte
- Gastroenterology Department, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
| | - David João
- Gastroenterology Department, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
| | - Teresa Freitas
- Gastroenterology Department, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
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Kagaya-Takeuchi S, Nagasawa T, Takeuchi Y, Takeda K, Joh K, Miyazaki M. Concomitant pleuritis and pericarditis developing during glucocorticoid therapy: a case report on granulomatosis with polyangiitis. CEN Case Rep 2022; 11:371-375. [PMID: 35107769 DOI: 10.1007/s13730-021-00678-8] [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: 03/31/2021] [Accepted: 12/15/2021] [Indexed: 11/30/2022] Open
Abstract
Granulomatosis with polyangiitis is an anti-neutrophil cytoplasmic antibody-associated vasculitis that manifests in various ways by affecting the small-sized vessels in multiple organs. Acute pleuritis and pericarditis are both rare among the different manifestations of granulomatosis with polyangiitis. The symptoms in each of the organs are often apparent at the time of diagnosis and tend to diminish with treatment. Organ damage and progression of the disease during treatment are uncommon. We encountered a patient with granulomatosis with polyangiitis who, after starting intravenous methylprednisolone pulse therapy, concurrently developed acute pleuritis and pericarditis. The patient was a 47-year-old Japanese man with myalgia in whom kidney dysfunction, proteinase 3-anti-neutrophil cytoplasmic antibody positivity, and a lung mass were detected. Granulomatosis with polyangiitis was diagnosed pathologically from a lung and a kidney biopsy. Acute pleuritis and pericarditis, which developed after the first course of intravenous methylprednisolone pulse therapy, both resolved following the second course. The present report indicates that secondary serositis such as pleuritis and pericarditis can develop in patients with granulomatosis with polyangiitis even during glucocorticoid therapy.
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Affiliation(s)
- Saeko Kagaya-Takeuchi
- Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Ishinomaki, Japan. .,Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan.
| | - Tasuku Nagasawa
- Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Ishinomaki, Japan.,Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Yoichi Takeuchi
- Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Ishinomaki, Japan.,Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Kenichiro Takeda
- Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Ishinomaki, Japan
| | - Kensuke Joh
- Division of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Mariko Miyazaki
- Division of Nephrology, Endocrinology and Vascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan
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Ahmad Y, Morawietz G, Ksouri H, Schefold JC, Zuercher P. Granulomatosis with polyangiitis (Wegener's) complicated by splenic rupture and severe acute respiratory distress syndrome: A case report. Clin Case Rep 2021; 9:e04369. [PMID: 34267899 PMCID: PMC8271247 DOI: 10.1002/ccr3.4369] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2021] [Revised: 05/08/2021] [Accepted: 05/11/2021] [Indexed: 12/02/2022] Open
Abstract
Even in the absence of disease-specific radiological signs of granulomatosis with polyangiitis (GPA), severe intrapulmonary GPA may be present. Rapidly establishing the diagnosis with a confirmatory biopsy is key to initiate lifesaving therapy.
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Affiliation(s)
- Yannis Ahmad
- Intensive Care UnitHFR Hôpital Cantonal FribourgFribourgSwitzerland
| | | | - Hatem Ksouri
- Intensive Care UnitHFR Hôpital Cantonal FribourgFribourgSwitzerland
| | - Joerg C. Schefold
- Department of Intensive Care MedicineInselspitalBern University HospitalUniversity of BernBernSwitzerland
| | - Patrick Zuercher
- Department of Intensive Care MedicineInselspitalBern University HospitalUniversity of BernBernSwitzerland
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Mollaeian A, Chan N, Aloor R, Iding JS, Arend LJ, Saeidabadi SHF, Haas CJ. ANCA-negative microscopic polyangiitis with diffuse alveolar hemorrhage masquerading as congestive heart failure. AUTOIMMUNITY HIGHLIGHTS 2021; 12:1. [PMID: 33407881 PMCID: PMC7788985 DOI: 10.1186/s13317-020-00143-z] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/19/2020] [Accepted: 10/07/2020] [Indexed: 11/10/2022]
Abstract
Background Microscopic polyangiitis (MPA) is a subtype of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), involving small and medium sized vessels, often affecting the kidneys and lungs. Anti-neutrophil cytoplasmic antibody (ANCA) is detected in up to 90% of cases of MPA and its detection helps guide diagnosis, however cases of ANCA-negative MPA have been reported, hence definitive diagnosis relies on tissue biopsy. Case report A 23-year-old man was evaluated for dyspnea and pleuritic chest pain, and found to have bilateral intra-alveolar opacities and hilar adenopathy. Diagnostic work up revealed positive anti-nuclear antibodies (ANA) and negative ANCA, which in the setting of a non-classical presentation, delayed diagnosis and appropriate treatment. Due to persistent symptoms and a high suspicion for autoimmune disease with pulmonary-renal syndrome, he underwent lung biopsy which revealed intra-alveolar hemorrhage and capillaritis indicative of microscopic polyangiitis (MPA). Surprisingly, kidney biopsy was not typical of classic MPA, but revealed less common features. Due to therapeutic noncompliance he was readmitted multiple times thereafter with rare complications of MPA such as acute pancreatitis and hemorrhagic pericardial effusion with tamponade. Conclusion This case serves as an important clinical reminder to consider AAV even in those with negative ANCA serologies and a high suspicion for pulmonary-renal syndrome. It also demonstrates the high morbidity in cases of diagnostic delay and inadequate treatment.
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Affiliation(s)
- Arash Mollaeian
- MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA.
| | - Nina Chan
- MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA
| | - Rohit Aloor
- MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA
| | - Jeffery S Iding
- Department of Pathology, MedStar Franklin Square Hospital, Baltimore, MD, USA
| | - Lois J Arend
- Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA
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Scurt FG, Bose K, Canbay A, Mertens PR, Chatzikyrkou C. [Acute kidney injury following acute pancreatitis (AP-AKI): Definition, Pathophysiology, Diagnosis and Therapy]. ZEITSCHRIFT FUR GASTROENTEROLOGIE 2020; 58:1241-1266. [PMID: 33291178 DOI: 10.1055/a-1255-3413] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Acute pancreatitis (AP) is the most frequent gastrointestinal cause for hospitalization and one of the leading causes of in-hospital deaths. Severe acute pancreatitis is often associated with multiorgan failure and especially with acute kidney injury (AKI). AKI can develop early or late in the course of the disease and is a strong determinator of outcome. The mortality in the case of dialysis-dependent AKI and acute pancreatitis raises exponentially in the affected patients. AP-induced AKI (AP-AKI) shows many similarities but also distinct differences to other causes of AKI occurring in the intensive care unit setting. The knowledge of the exact pathophysiology can help to adjust, control and improve therapeutic approaches to the disease. Unfortunately, there are only a few studies dealing with AP and AKI.In this review, we discuss recent data about pathogenesis, causes and management of AP-AKI in patients with severe acute pancreatitis and exploit in this regard the diagnostic and prognostic potential of respective newer serum and urine markers.
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Affiliation(s)
- Florian Gunnar Scurt
- Klinik für Nieren- und Hochdruckerkrankungen, Diabetologie und Endokrinologie, Medizinische Fakultät der Otto-von-Guericke-Universität, Magdeburg, Deutschland.,Health Campus Immunology, Infectiology and Inflammation, Otto-von-Guericke-University, Magdeburg, Germany
| | - Katrin Bose
- Health Campus Immunology, Infectiology and Inflammation, Otto-von-Guericke-University, Magdeburg, Germany.,Universitätsklinik für Gastroenterologie, Hepatologie und Infektiologie, Medizinische Fakultät der Otto-von-Guericke-Universität, Otto-von-Guericke-Universität, Magdeburg, Deutschland
| | - Ali Canbay
- Ruhr-Universität Bochum, Medizinische Klinik, Universitätsklinikum Knappschaftskrankenhaus Bochum GmbH, Bochum, Deutschland
| | - Peter R Mertens
- Klinik für Nieren- und Hochdruckerkrankungen, Diabetologie und Endokrinologie, Medizinische Fakultät der Otto-von-Guericke-Universität, Magdeburg, Deutschland.,Health Campus Immunology, Infectiology and Inflammation, Otto-von-Guericke-University, Magdeburg, Germany
| | - Christos Chatzikyrkou
- Klinik für Nieren- und Hochdruckerkrankungen, Diabetologie und Endokrinologie, Medizinische Fakultät der Otto-von-Guericke-Universität, Magdeburg, Deutschland.,Health Campus Immunology, Infectiology and Inflammation, Otto-von-Guericke-University, Magdeburg, Germany
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Gercik O, Karasu S, Solmaz D, Soypacaci Z, Cakalagaoglu F, Akar S. Splenic infarction is not rare in granulomatosis with polyangiitis. Clin Rheumatol 2020; 39:1929-1934. [PMID: 32107662 DOI: 10.1007/s10067-020-04993-w] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2019] [Revised: 02/10/2020] [Accepted: 02/14/2020] [Indexed: 10/25/2022]
Abstract
Splenic involvement is rarely reported in patients with ANCA-associated vasculitides (AAVs), particularly in those with granulomatosis with polyangiitis (GPA) and is in fact considered to be underestimated. We aimed to investigate the frequency of splenic lesions-mainly infarction-and related factors in patients with AAVs. Patients with AAV whose abdominal or thoracic computed tomographies (CTs) were performed at or after diagnosis were included in the study. CT images were examined for splenic lesions. Overall, 69 patients (median age at diagnosis 54 [IQR 24] years; 55% with GPA, 29% with microscopic polyangiitis, and 16% with renal-limited disease) were included in the analysis. Splenic pathologies were detected in 19 (28%) patients; 12/19 (63%) splenomegaly and 7/19 (37%) splenic infarction. All patients with splenic infarction exhibited GPA with PR3-ANCA-positive serology. Three of these seven patients had autosplenectomy. Patients with splenic infarction were younger at diagnosis (p = 0.018) with also significantly higher ear-nose-throat (ENT) (%100 vs 37; p = 0.002) and eye involvement (%50 vs %12; p = 0.044) than patients without splenic infarction. Splenic pathologies are not rare in AAVs. Furthermore, infarction can help separate GPA from MPA. In young patients with GPA, particularly those with ENT and eye involvements, physicians should consider splenic infarction.Key Points• Splenic infarction is more common than previously thought in ANCA-associated vasculitides, particularly in granulomatosis with polyangiitis.• Detecting splenic infarction can help differentiate granulomatosis with polyangiitis from other subgroups.
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Affiliation(s)
- Onay Gercik
- Faculty of Medicine, Department of Rheumatology, Izmir Katip Celebi University, Karabaglar, 35360, Izmir, Turkey
| | - Sebnem Karasu
- Faculty of Medicine, Department of Radiology, Izmir Katip Celebi University, Izmir, Turkey
| | - Dilek Solmaz
- Faculty of Medicine, Department of Rheumatology, Izmir Katip Celebi University, Karabaglar, 35360, Izmir, Turkey
| | - Zeki Soypacaci
- Faculty of Medicine, Department of Nephrology, Izmir Katip Celebi University, Izmir, Turkey
| | - Fulya Cakalagaoglu
- Faculty of Medicine, Department of Pathology, Izmir Katip Celebi University, Izmir, Turkey
| | - Servet Akar
- Faculty of Medicine, Department of Rheumatology, Izmir Katip Celebi University, Karabaglar, 35360, Izmir, Turkey.
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