KEGG   DISEASE: Eosinophilic granulomatosis with polyangiitis
Entry
H01468                      Disease                                
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
Cardiovascular disease
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Vascular diseases
   H01468  Eosinophilic granulomatosis with polyangiitis
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Nonorgan specific systemic autoimmune disorders
   4A44  Vasculitis
    H01468  Eosinophilic granulomatosis with polyangiitis
Related
pathway
hsa04672 Intestinal immune network for IgA production   
hsa04612 Antigen processing and presentation   
hsa04658 Th1 and Th2 cell differentiation   
hsa04659 Th17 cell differentiation   
hsa04640 Hematopoietic cell lineage   
Gene
HLA-DRB1 (polymorphism) [HSA:3123] [KO:K06752]
HLA-DRB4 (polymorphism) [HSA:3126] [KO:K06752]
IL10 (polymorphism) [HSA:3586] [KO:K05443]
Other DBs
ICD-11: 4A44.A2
ICD-10: M30.1
MeSH: D015267
Reference
PMID:25404930 (gene, marker)
  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
Reference
  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
Reference
  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
Reference
  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
Reference
  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
Reference
PMID:25709744 (drug)
  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
Reference
PMID:25467294 (drug)
  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
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