Progress in diagnosis and treatment of pediatric microscopic polyangiitis
10.3760/cma.j.issn.2095-428X.2017.05.004
- VernacularTitle:儿童显微镜下多血管炎诊治进展
- Author:
Liangzhong SUN
;
Haiyan WANG
- Keywords:
Microscopic polyangiitis;
Anti-neutrophil cytoplasmic antibody;
Crescentic glomerulonephritis
- From:
Chinese Journal of Applied Clinical Pediatrics
2017;32(5):333-337
- CountryChina
- Language:Chinese
-
Abstract:
Microscopic polyangiitis (MPA) is an anti-neutrophil cytoplasmic antibody (ANCA) associated systemic small vessel vasculitis and is uncommon in children.Pathologically characterized by paucity immune deposition,fibroid necrosis and crescent formation in glomeruli.MPA is a multi-organ involvement disease.Renal is the mostly involved and commonly manifested as aggressive glomerulonephritis.Lung is the most common involved extrarenal organ.Most MPA patients have positive myeloperoxidase-ANCA and positive perinuclear-ANCA.The onset of MPA is usually obscure,which makes early diagnosis difficult.Detection of ANCA is performed in order to discriminate suspected MPA patients earlier.Confirmed diagnosis relies on pathology.Early standardized treatment is a key factor in prognosis.Standard inductive treatment is currently the combination of corticosteroids with the cytotoxic agent cyclophosphamide.Azathioprine is suggested as the first-choice medication in maintenance therapy.Bio-agents,such as Rituximab,have shown good curative effect both in the inductive treatment and maintenance therapy.
