| H01468 | |
| H number | H01468 |
| Name | Eosinophilic granulomatosis with polyangiitis; Churg-Strauss syndrome |
| Description | 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. |
| Category | Immune system disease |
| Network | - |
| Gene | HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752] HLA-DRB4 (polymorphism) [HSA:3126] [KO:K06752] IL10 (polymorphism) [HSA:3586] [KO:K05443] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | - |
| Other DBs | ICD-11: 4A44.A2 ICD-10: M30.1 MeSH: D015267 |
| Reference | PMID:25404930 AUTHORS Gioffredi A, Maritati F, Oliva E, Buzio C TITLE Eosinophilic granulomatosis with polyangiitis: an overview. JOURNAL Front Immunol 5:549 (2014) DOI:10.3389/fimmu.2014.00549 PMID:25500434 AUTHORS Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M TITLE Churg-Strauss syndrome. JOURNAL Autoimmun Rev 14:341-8 (2015) DOI:10.1016/j.autrev.2014.12.004 PMID:24072416 AUTHORS Jennette JC TITLE Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. JOURNAL Clin Exp Nephrol 17:603-6 (2013) DOI:10.1007/s10157-013-0869-6 PMID:17989571 AUTHORS Bacciu A, Buzio C, Giordano D, Pasanisi E, Vincenti V, Mercante G, Grasselli C, Bacciu S TITLE Nasal polyposis in Churg-Strauss syndrome. JOURNAL Laryngoscope 118:325-9 (2008) DOI:10.1097/MLG.0b013e318159889d PMID:18512809 AUTHORS Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT TITLE Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis. JOURNAL Arthritis Rheum 58:1839-48 (2008) DOI:10.1002/art.23496 PMID:25709744 AUTHORS Mukherjee M, Sehmi R, Nair P TITLE Anti-IL5 therapy for asthma and beyond. JOURNAL World Allergy Organ J 7:32 (2014) DOI:10.1186/1939-4551-7-32 PMID:25467294 AUTHORS Mohammad AJ, Hot A, Arndt F, Moosig F, Guerry MJ, Amudala N, Smith R, Sivasothy P, Guillevin L, Merkel PA, Jayne DR TITLE Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). JOURNAL Ann Rheum Dis 75:396-401 (2016) DOI:10.1136/annrheumdis-2014-206095 |