KEGG   DISEASE: IgA vasculitis
Entry
H01584                      Disease                                
Name
IgA vasculitis;
Henoch-Schonlein purpura
Description
IgA vasculitis (IgAV), also known as Henoch-Schonlein purpura (HSP), is the most common systemic small vessel vasculitis in childhood with clinical characteristics of non-thrombocytopenic palpable purpura, arthritis, and involvement of internal organs such as gastrointestine (GI) and kidney. IgAV has been associated with a history of preceding infections, especially upper respiratory tract infection. In addition, other characteristics of IgAV include the deposition of IgA and C3 in small vessel walls, polymorphonuclear neutrophil infiltration around the vessel and in vessel walls, and increased serum levels of IgA and proinflammatory cytokines at the acute stage. Combined, IgAV is regarded as a specific immune-mediated entity induced by environmental factors, particularly infections. Clinically, since there are no disease-specific laboratory abnormalities, IgAV is currently diagnosed based on symptoms and signs and histopathological findings. Treatment is supportive because IgAV is usually self-limiting except for serious GI or renal involvement.
Category
Cardiovascular disease
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Hematologic diseases
   H01584  IgA vasculitis
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Nonorgan specific systemic autoimmune disorders
   4A44  Vasculitis
    H01584  IgA vasculitis
Other DBs
ICD-11: 4A44.92
ICD-10: D69.0
MeSH: D011695
Reference
  Authors
Yang YH, Yu HH, Chiang BL
  Title
The diagnosis and classification of Henoch-Schonlein purpura: an updated review.
  Journal
Autoimmun Rev 13:355-8 (2014)
DOI:10.1016/j.autrev.2014.01.031
Reference
  Authors
He X, Yu C, Zhao P, Ding Y, Liang X, Zhao Y, Yue X, Wu Y, Yin W
  Title
The genetics of Henoch-Schonlein purpura: a systematic review and meta-analysis.
  Journal
Rheumatol Int 33:1387-95 (2013)
DOI:10.1007/s00296-012-2661-4
LinkDB

» Japanese version

DBGET integrated database retrieval system