| H01468 | |
| H番号 | H01468 |
| 名称 | 好酸球性多発血管炎性肉芽腫症; チャーグストラウス症候群 |
| 概要 | Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitic disorder of unknown etiology that affects small-to-medium-size blood vessels. This disease has been called Churg-Strauss syndrome (CSS) for many years, and renamed eosinophilic granulomatosis with polyangiitis (EGPA) in 2012. EGPA is characterized by asthma, hypereosinophilia, and extravascular eosinophilic granulomas. Clinically, three phases may be distinguished. The prodromal phase may persist for many years, consisting of asthma possibly associated with allergic rhinitis and often complicated by recurrent rhinosinusitis and nasal polyps. The second phase is characterized by peripheral blood eosinophilia or eosinophilic tissue infiltrates. The third phase is dominated by manifestations resulting from systemic vasculitis. Vasculitis commonly affects the skin, nerves, gastrointestinal tract, and heart. It can be serious and life-threatening. EGPA pathogenesis is not well known. The disease is probably the result of a complex interaction in which genetically and environmental factors lead to an inflammatory response whose principal players are eosinophils, T, and B lymphocytes. HLA-DRB1 and DRB4 alleles and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative. |
| カテゴリ | 免疫系疾患 |
| ネットワーク | - |
| 病因遺伝子 | HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752] HLA-DRB4 (polymorphism) [HSA:3126] [KO:K06752] IL10 (polymorphism) [HSA:3586] [KO:K05443] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | メポリズマブ [DR:D04923] メチルプレドニゾロンコハク酸エステルナトリウム [DR:D00751] アザチオプリン [DR:D00238] シクロホスファミド水和物 [DR:D00287] 乾燥スルホ化人免疫グロブリン [DR:D08786] |
| コメント | - |
| リンク | ICD-11: 4A44.A2 ICD-10: M30.1 MeSH: D015267 |
| 文献 | PMID:25404930 著者 Gioffredi A, Maritati F, Oliva E, Buzio C タイトル Eosinophilic granulomatosis with polyangiitis: an overview. 雑誌 Front Immunol 5:549 (2014) DOI:10.3389/fimmu.2014.00549 PMID:25500434 著者 Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M タイトル Churg-Strauss syndrome. 雑誌 Autoimmun Rev 14:341-8 (2015) DOI:10.1016/j.autrev.2014.12.004 PMID:24072416 著者 Jennette JC タイトル Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. 雑誌 Clin Exp Nephrol 17:603-6 (2013) DOI:10.1007/s10157-013-0869-6 PMID:17989571 著者 Bacciu A, Buzio C, Giordano D, Pasanisi E, Vincenti V, Mercante G, Grasselli C, Bacciu S タイトル Nasal polyposis in Churg-Strauss syndrome. 雑誌 Laryngoscope 118:325-9 (2008) DOI:10.1097/MLG.0b013e318159889d PMID:18512809 著者 Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT タイトル Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis. 雑誌 Arthritis Rheum 58:1839-48 (2008) DOI:10.1002/art.23496 PMID:25709744 著者 Mukherjee M, Sehmi R, Nair P タイトル Anti-IL5 therapy for asthma and beyond. 雑誌 World Allergy Organ J 7:32 (2014) DOI:10.1186/1939-4551-7-32 PMID:25467294 著者 Mohammad AJ, Hot A, Arndt F, Moosig F, Guerry MJ, Amudala N, Smith R, Sivasothy P, Guillevin L, Merkel PA, Jayne DR タイトル Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). 雑誌 Ann Rheum Dis 75:396-401 (2016) DOI:10.1136/annrheumdis-2014-206095 |