Progress in diagnosis and treatment of pediatric microscopic polyangiitis / 中华实用儿科临床杂志
Chinese Journal of Applied Clinical Pediatrics
;
(24): 333-337, 2017.
Artigo
em Chinês
| WPRIM
| ID: wpr-510846
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Tipo de estudo:
Estudo de rastreamento
Idioma:
Chinês
Revista:
Chinese Journal of Applied Clinical Pediatrics
Ano de publicação:
2017
Tipo de documento:
Artigo
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