KEGG   DISEASE: Microscopic polyangiitis
Entry
H01658                      Disease                                
Name
Microscopic polyangiitis
Description
Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. MPA predominantly affects small-calibre blood vessels and is associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). The association with ANCAs originally defined the group of ANCA-associated vasculitides, comprising granulomatosis with polyangitis (GPA, formerly known as Wegener granulomatosis) [DS:H01655], microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) [DS:H01468], which have different frequencies of ANCA-positivity. ANCA in MPA are predominantly directed against myeloperoxidase (MPO-ANCA) but may, in a minority of patients, be directed against proteinase 3 (PR3-ANCA). Not all patients, however, have ANCA. MPA is clinically characterized by small-vessel vasculitis primarily affecting the kidneys and the lungs but other organs may be involved as well. Renal involvement, which can be the only manifestation, is clinically apparent as rapidly progressive glomerulonephritis and histopathologically as pauci-immune necrotizing and crescentic glomerulonephritis. Diagnosis is mainly established by clinical manifestations, computed tomography (TC), ANCA antibody detection, and renal and pulmonary biopsy. The introduction of aggressive immunosuppressive treatment has substantially improved the prognosis. The standardized therapeutic regimen is based on cyclophosphamide and corticosteroids. Using this regimen, remission can be achieved in most of the patients. Rituximab may represent an important alternative to cyclophosphamide for patients who may not respond adequately to antimetabolite therapies.
Category
Nervous system disease; Cardiovascular disease; Immune system disease
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Vascular diseases
   H01658  Microscopic polyangiitis
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Nonorgan specific systemic autoimmune disorders
   4A44  Vasculitis
    H01658  Microscopic polyangiitis
Drug
Rituximab [DR:D02994]
Other DBs
ICD-11: 4A44.A0
ICD-10: M31.7
MeSH: D055953
Reference
PMID:25992801 (description, drug)
  Authors
Greco A, De Virgilio A, Rizzo MI, Gallo A, Magliulo G, Fusconi M, Ruoppolo G, Tombolini M, Turchetta R, de Vincentiis M
  Title
Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches.
  Journal
Autoimmun Rev 14:837-44 (2015)
DOI:10.1016/j.autrev.2015.05.005
Reference
  Authors
Kallenberg CG
  Title
The diagnosis and classification of microscopic polyangiitis.
  Journal
J Autoimmun 48-49:90-3 (2014)
DOI:10.1016/j.jaut.2014.01.023
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