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 extrare-nal 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. Key words: Microscopic polyangiitis; Anti-neutrophil cytoplasmic antibody; Crescentic glomerulonephritis
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