Pintos-Morell G, Roca-Comas A, Naranjo M A, Tural C, Abad E, Javier G, Prats J
Department of Paediatrics, University Hospital Germans Trias i Pujol, Badalona, Spain.
Eur J Pediatr. 1993 Jun;152(6):473-5. doi: 10.1007/BF01955052.
We present a 13-year-old boy with a rapidly progressive glomerulonephritis and pulmonary haemorrhage with perinuclear anti-neutrophil cytoplasmic auto-antibodies (pANCA) corresponding to anti-myeloperoxidase antibodies. The diagnosis of microscopic polyarteritis was made on the basis of the clinical features, the positivity of pANCA, and the histological finding of a pauci-immune crescentic glomerulonephritis. He responded excellently to corticosteroids and cyclophosphamide therapy and complete clinical remission persists 1 year after withdrawal of treatment. We emphasize the usefulness of ANCA antibody assays to establish a prompt diagnosis and adequate treatment in systemic vasculitis in children.
我们报告一名13岁男孩，患有快速进展性肾小球肾炎和肺出血，伴有与抗髓过氧化物酶抗体相对应的核周抗中性粒细胞胞浆自身抗体（pANCA）。根据临床特征、pANCA阳性以及寡免疫性新月体性肾小球肾炎的组织学表现，做出了显微镜下多动脉炎的诊断。他对皮质类固醇和环磷酰胺治疗反应良好，停药1年后仍保持完全临床缓解。我们强调ANCA抗体检测在儿童系统性血管炎中对于快速诊断和适当治疗的有用性。
