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Chang C, Wang Y, Shi W, Xu H, Huang X, Jiao Y. Is conservative management a safe approach for patients with acute acalculous cholecystitis presenting with an acute abdomen? Medicine (Baltimore) 2023; 102:e34662. [PMID: 37656996 PMCID: PMC10476726 DOI: 10.1097/md.0000000000034662] [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: 03/25/2023] [Accepted: 07/19/2023] [Indexed: 09/03/2023] Open
Abstract
Patients with acute acalculous cholecystitis (AAC) often present with acute abdominal symptoms. However, recent clinical studies have suggested that some patients with AAC and an acute abdomen, especially when caused by viruses or rheumatic disease, may not require cholecystectomy and that conservative treatment is adequate. Whether cholecystectomy is superior to conservative treatment for patients with AAC presenting with a severe acute abdomen is still uncertain. This was a case series study of AAC-related literature published between 1960 and 2022. In total, 171 cases (104 viral infection-associated AAC and 67 rheumatic disease-associated AAC) were included. The prognoses of patients receiving cholecystectomy or conservative treatment were compared. To account for confounding factors, etiological stratification and logistic regression were performed. The prognosis was similar for patients undergoing cholecystectomy and conservative treatment (P value .364), and virus infection-associated AAC had a better prognosis than rheumatic disease-associated AAC (P value .032). In patients with AAC caused by viruses or rheumatic disease, the acute abdomen can be adequately managed by conservative treatment of the underlying etiology and does not mandate surgical intervention.
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Affiliation(s)
- Chuheng Chang
- Department of General Practice (General Internal Medicine), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Youyang Wang
- Department of General Practice (General Internal Medicine), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Wen Shi
- Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Haifeng Xu
- Department of Liver Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Xiaoming Huang
- Department of General Practice (General Internal Medicine), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Yang Jiao
- Department of General Practice (General Internal Medicine), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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2
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Sato M, Yatomi M, Wakamatsu I, Uno S, Hanazato C, Masuda T, Yamaguchi K, Aoki-Saito H, Kasahara N, Miura Y, Tsurumaki H, Hara K, Koga Y, Sunaga N, Okada T, Ikota H, Hisada T, Maeno T. Effective treatment with mepolizumab in a patient with severe eosinophilic granulomatosis with polyangiitis complicated with small intestine perforation. Respir Med Case Rep 2023; 43:101818. [PMID: 36950026 PMCID: PMC10025129 DOI: 10.1016/j.rmcr.2023.101818] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2022] [Revised: 01/29/2023] [Accepted: 02/08/2023] [Indexed: 02/27/2023] Open
Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is systemic vasculitis caused by eosinophilia affecting small to medium-sized blood vessels, which damages the organs. Antineutrophil cytoplasmic antibody-associated vasculitis EGPA treatment guidelines added anti-interleukin-5 antibody mepolizumab to the standard treatment protocol for active-non-severe EGPA based on the MIRRA study. Nevertheless, the role of mepolizumab in treating patients with active severe EGPA has not been established. We treated a patient with EGPA complicated with small intestine perforation using steroid pulse intravenous, high-dose glucocorticoids, intravenous high-dose immunoglobulin therapy, and mepolizumab without immunosuppression agents; the patient went into remission, suggesting that mepolizumab is an effective therapeutic agent that could lead to remission in severe EGPA.
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Affiliation(s)
- Mari Sato
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Masakiyo Yatomi
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
- Corresponding author.
| | - Ikuo Wakamatsu
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Shogo Uno
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Chiharu Hanazato
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Tomomi Masuda
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Koichi Yamaguchi
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Haruka Aoki-Saito
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Norimitsu Kasahara
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Yosuke Miura
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Hiroaki Tsurumaki
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Kenichiro Hara
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Yasuhiko Koga
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Noriaki Sunaga
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Takuhisa Okada
- Department of General Surgical Science, Gunma University Graduate School of Medicine, Gunma, 3-39-22, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
| | - Hayato Ikota
- Department of Diagnostic Pathology, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371-8511, Japan
| | - Takeshi Hisada
- Gunma University Graduate School of Health Sciences, 3-39-22, Showa-machi, Maebashi, Gunma, 371-8514, Japan
| | - Toshitaka Maeno
- Division of Allergy and Respiratory Medicine, Integrative Center of Internal Medicine, Gunma University Hospital, 3-39-15, Showa-machi, Maebashi, Gunma, 371- 8511, Japan
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3
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Ozaka S, Kodera T, Tsutsumi K, Fukuda M, Iwao M, Hirashita Y, Takahashi H, Fukuda K, Okamoto K, Arakawa M, Ogawa R, Endo M, Mizukami K, Okimoto T, Kamiyama N, Kodama M, Kobayashi T, Murakami K. Acute Cholecystitis Associated with Eosinophilic Granulomatosis with Polyangiitis that Responded to Glucocorticoid Therapy. Intern Med 2022; 62:1501-1506. [PMID: 36171126 DOI: 10.2169/internalmedicine.0437-22] [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] [Indexed: 11/06/2022] Open
Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic disorder characterized by tissue eosinophilic infiltration and vasculitis. Although EGPA causes multiple organ damage, it causes cholecystitis less frequently. We herein report a case of acute cholecystitis associated with EGPA in which successful treatment with glucocorticoid therapy allowed surgery to be avoided. EGPA can present as acute cholecystitis. It is important not to overlook acute cholecystitis associated with EGPA in patients with abdominal pain with peripheral eosinophilia. Furthermore, in cases of mild cholecystitis associated with EGPA that are diagnosed preoperatively, cholecystectomy might be avoided with conservative glucocorticoid treatment.
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Affiliation(s)
- Sotaro Ozaka
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
- Department of Infectious Disease Control, Faculty of Medicine, Oita University, Japan
| | - Takamoto Kodera
- Department of Internal Medicine, Saiki Central Hospital, Japan
| | - Koshiro Tsutsumi
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Masahide Fukuda
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Masao Iwao
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Yuka Hirashita
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Haruhiko Takahashi
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Kensuke Fukuda
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Kazuhisa Okamoto
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Mie Arakawa
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Ryo Ogawa
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Mizuki Endo
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Kazuhiro Mizukami
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Tadayoshi Okimoto
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Naganori Kamiyama
- Department of Infectious Disease Control, Faculty of Medicine, Oita University, Japan
| | - Masaaki Kodama
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
| | - Takashi Kobayashi
- Department of Infectious Disease Control, Faculty of Medicine, Oita University, Japan
| | - Kazunari Murakami
- Department of Gastroenterology, Faculty of Medicine, Oita University, Japan
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Salsman ML, Nordberg HO, Wittchen HU, Klotsche J, Mühlig S, Riedel O, Ritz T. Extrapulmonary symptoms of patients with asthma treated in specialist pulmonary care. J Psychosom Res 2021; 148:110538. [PMID: 34174713 DOI: 10.1016/j.jpsychores.2021.110538] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/25/2020] [Revised: 05/27/2021] [Accepted: 05/28/2021] [Indexed: 10/21/2022]
Abstract
OBJECTIVE Patients seeking treatment for their asthma are most likely motivated by a change in their experience of symptoms, but primary complaints are not always related to the pulmonary system. This study aimed to determine the frequency of such extrapulmonary symptoms in asthma outpatients and their association with psychopathology and asthma outcomes. METHODS This cross-sectional study utilized data collected as part of a nationwide, clinical-epidemiological study. The final sample of 572 asthma patients represented all levels of asthma control and severity. Information on demographics and respiratory function was obtained from physicians' documentation. Symptoms were explored using a standardized checklist. RESULTS Primary symptoms reported by asthma patients were not necessarily airway-related. Patients reported feeling at least occasionally "tired" (72.1%) and "exhausted" (66.8%) more than any other asthma symptom. Hyperventilation and mood symptoms were experienced by 34.4-42.6% of patients. Anxiety or depression diagnoses indicated higher scores in all symptom domains. Controlling for asthma-related factors and psychopathology, fatigue had a small but significant effect on both asthma-related quality of life (AQLQ) (rsp2 = 0.02, P < .001) and asthma control (rsp2 = 0.01, P = .003). Mood symptoms also showed a small but significant effect on AQLQ (rsp2 = 0.02, P < .001). CONCLUSION Findings suggest that extrapulmonary symptoms are endorsed more frequently than previously reported. Symptoms nonspecific to asthma can play a substantial role in clinical presentation and exclusive focus on airway symptoms may miss important information related to patients' well-being. Surveillance of extrapulmonary symptoms alongside pulmonary function is warranted for an integrated medicine approach to asthma management.
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Affiliation(s)
- Margot L Salsman
- Department of Psychology, Southern Methodist University, Dallas, TX, USA
| | - Hannah O Nordberg
- Department of Psychology, Southern Methodist University, Dallas, TX, USA
| | - Hans-Ulrich Wittchen
- Institute of Clinical Psychology and Psychotherapy, Technische Universität Dresden, Dresden, Germany; Department of Psychiatry and Psychotherapy, Ludwig-Maximilians-University (LMU) Muenchen, Germany
| | - Jens Klotsche
- Deutsches Rheumaforschungszentrum, Ein Leibniz-Zentrum, Berlin, Germany
| | - Stephan Mühlig
- Chemnitz University of Technology, Department of Psychology, Chemnitz, Germany
| | - Oliver Riedel
- Department of Clinical Epidemiology, Leibniz-Institute for Prevention Research and Epidemiology - BIPS, Bremen, Germany
| | - Thomas Ritz
- Department of Psychology, Southern Methodist University, Dallas, TX, USA.
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5
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Vélez-Velázquez MD, Busteros-Moraza JI, Alonso-Riaño M, Martínez-Onsurbe MP. Colecistitis aguda alitiásica como manifestación inicial de una granulomatosis eosinofílica con poliangeítis (Churg-Strauss). A propósito de un caso. REVISTA ESPAÑOLA DE PATOLOGÍA 2017; 50:257-261. [DOI: 10.1016/j.patol.2016.04.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2025]
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6
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He JN, Tian Z, Yao X, Li HY, Yu Y, Liu Y, Liu JG. Multiple perforations and fistula formation following corticosteroid administration: A case report. World J Clin Cases 2017; 5:67-72. [PMID: 28255551 PMCID: PMC5314264 DOI: 10.12998/wjcc.v5.i2.67] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/20/2016] [Revised: 08/28/2016] [Accepted: 12/14/2016] [Indexed: 02/05/2023] Open
Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We report a patient with EPGA treated with high-dose steroid who underwent emergency surgery for intestinal perforations. We performed a simple repair of the 11 perforations. Intestinal fistulas developed 8 d postoperatively; they healed well after 60 d of continuous washing and negative pressure suction. The clinical data of 14 additional patients with EGPA or Churg-Strauss syndrome complicated with gastrointestinal perforation, which were reported from 1996 to 2014, were also collected and compared. The formation of multiple perforations and fistulas following high dosage steroid administration can have a good outcome with appropriate management. Meticulous attention to abdominal symptoms and appropriate interventions can result in timely management. Corticosteroid administration remains a very important perioperative procedure for EPGA.
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7
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Ye L, Lu X, Xue J. Eosinophilic granulomatosis with polyangiitis complicated by cholecystitis: a case report and review of the literature. Clin Rheumatol 2014; 35:259-63. [PMID: 24515868 DOI: 10.1007/s10067-014-2521-6] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2014] [Revised: 01/28/2014] [Accepted: 01/29/2014] [Indexed: 11/30/2022]
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8
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Rolla G, Guida G, Heffler E. Churg–Strauss syndrome: still a clinical challenge. Expert Rev Clin Immunol 2014; 3:833-7. [DOI: 10.1586/1744666x.3.6.833] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
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9
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Lee WJ, Hwang JW, Kim E, Yune S, Ha JM, Yoon N, Lee BJ, Choi DC. Churg-Strauss syndrome presenting as acute acalculous cholecystitis. ALLERGY ASTHMA & RESPIRATORY DISEASE 2013. [DOI: 10.4168/aard.2013.1.4.388] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Affiliation(s)
- Woo Joo Lee
- Department of Internal Medicine, Samsung Medical Center, Seoul, Korea
| | - Ji-Won Hwang
- Department of Internal Medicine, Samsung Medical Center, Seoul, Korea
| | - Eun Kim
- Department of Internal Medicine, Samsung Medical Center, Seoul, Korea
| | - Sehyo Yune
- Department of Internal Medicine, Samsung Medical Center, Seoul, Korea
| | - Jung Min Ha
- Department of Internal Medicine, Samsung Medical Center, Seoul, Korea
| | - Nara Yoon
- Department of Pathology, Samsung Medical Center, Seoul, Korea
| | - Byung Jae Lee
- Department of Internal Medicine, Samsung Medical Center, Seoul, Korea
- Department of Medicine, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Dong Chull Choi
- Department of Internal Medicine, Samsung Medical Center, Seoul, Korea
- Department of Medicine, Sungkyunkwan University School of Medicine, Seoul, Korea
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10
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Singh R, Singh D, Abdou N. Churg-Strauss syndrome presenting as acute abdomen: are gastrointestinal manifestations an indicator of poor prognosis? Int J Rheum Dis 2010; 12:161-5. [PMID: 20374336 DOI: 10.1111/j.1756-185x.2009.01399.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023]
Abstract
Churg Strauss syndrome (CSS) is a rare syndrome of vasculitis of the small to medium vessels, characterized by a triad of asthma, eosinophilia and atopic sinusitis. Although asthma is the most common manifestation, it is rarely associated with poor prognosis. Severe gastrointestinal tract manifestations are associated with worse outcomes. Patients with CSS at initial presentation, like our patient, may not meet all four American College of Rheumatology criteria to establish a diagnosis; however, clinicians should have a high index of suspicion to recognize the disease early in its course and treat aggressively, thereby decreasing the mortality and morbidity associated with this disease.
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Affiliation(s)
- Reetu Singh
- Internal Medicine, University of Missouri Kansas City, Kansas City, MO, USA.
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11
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Szwarc D, Veillon F, Moser T, Averous G, De Blay F, Riehm S. [Churg-Strauss syndrome under omalizumab treatment: a rare visceral manifestation]. ACTA ACUST UNITED AC 2010; 90:1737-9. [PMID: 19953062 DOI: 10.1016/s0221-0363(09)73273-1] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Affiliation(s)
- D Szwarc
- Service de Radiologie, Hôpital de Hautepierre, Strasbourg Cedex, France.
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12
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Sherlock Holmes in the ER (the case of red and the head). Surv Ophthalmol 2009; 54:128-34. [PMID: 19171214 DOI: 10.1016/j.survophthal.2008.10.017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Abstract
A 58-year-old woman presented with a problem with her peripheral vision. Computed tomography scan showed an occipital hemorrhagic stroke. She subsequently suffered gastrointestinal bleeding and at surgery biopsy of a portion of the middle colic artery aneurysm revealed changes consistent with polyarteritis nodosa.
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Kim JE, Kim KU, Park HK, Jeon DS, Kim YS, Lee MK, Park SK. A Case of Churg-Strauss Syndrome with Diffuse Alveolar Hemorrhage Presenting as Acute Acalculous Cholecystitis. Tuberc Respir Dis (Seoul) 2009. [DOI: 10.4046/trd.2009.66.3.225] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
Affiliation(s)
- Ji Eun Kim
- Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea
| | - Ki Uk Kim
- Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea
| | - Hye-Kyung Park
- Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea
| | - Doo Soo Jeon
- Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea
| | - Yun Sung Kim
- Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea
| | - Min Ki Lee
- Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea
| | - Soon Kew Park
- Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea
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14
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El-Gamal Y. Churg-strauss syndrome in the pediatric age group. World Allergy Organ J 2008; 1:34-40. [PMID: 23283308 PMCID: PMC3650976 DOI: 10.1097/wox.0b013e3181626fde] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2007] [Accepted: 11/16/2007] [Indexed: 12/15/2022] Open
Abstract
The rate of reporting of childhood Churg-Strauss syndrome (CSS) has increased lately because of either increased awareness to the disease or a real increase in incidence. It is defined as one of the antineutrophil cytoplasmic antibody-associated vasculitides, but the antineutrophil cytoplasmic antibody positivity is less reported in pediatric cases. The cause of CSS remains unknown. Several lines of evidence suggest genetic predisposition, which may entail inherited tendency to dysregulation of the cellular immune system. With the addition of leukotriene receptor antagonists to the treatment regimen of asthma, an association to CSS was presumed. However, the nature of this relationship remains to be elucidated. In addition, some environmental factors seem to provoke transient effects that resemble the disease. Patients' symptoms are defined by various degrees of eosinophilic inflammation and necrotizing vasculitis, which may affect any organ. Three clinical stages have been described in the clinical evolution of CSS: prodromal phase involving allergic rhinitis and asthma (usually without family history of atopy), a second phase that involves peripheral eosinophilia and eosinophilic tissue infiltration, and the hallmark of the final phase is systemic vasculitis. Pulmonary disease is a central feature of pediatric CSS, but other manifestations include skin lesions, testicular pain, hypertension, seizures, and nephropathy. More subtle presentations in children include cervical lymphadenopathy, acute abdominal pain, deep venous thrombosis, oral ulceration, multiple colonic ulcers, chorea, bilateral optic neuropathy, and retinal artery occlusions. Churg-Strauss syndrome patients usually respond well to corticosteroid therapy. Several trials reported additional benefit from cyclophosphamide, azathioprine, and methotrexate, whereas the therapeutic effects of etanercept, plasma exchange, and intravenous immunoglobulin therapy are controversial. The relapse rate is approximately 25% to 30%, but corticosteroids have significantly increased survival, which now approaches greater than 75% at 5 years. However, there is limited information about survival or long-term outcome in childhood.
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Affiliation(s)
- Yehia El-Gamal
- The Pediatric Allergy and Immunology Unit, Ain Shams University, Cairo, Egypt. 98 Mohamed Farid St, Cairo 11111, Egypt
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