Besbas N, Duzova A, Topaloglu R, Gok F, Ozaltin F, Ozen S, Bakkaloglu A
Hacettepe University Faculty of Medicine, Ankara, Turkey.
Clin Rheumatol. 2001;20(4):293-6. doi: 10.1007/pl00011205.
Henoch-Schönlein purpura (HSP) is the most common vasculitis in children. It is a multisystemic disease but pulmonary haemorrhage is extremely rare. We present the case of a 6-year-old boy with Henoch-Schönlein purpura, pulmonary haemorrhage and severe renal involvement. The patient responded to a combination of intravenous methylprednisolone and cyclophosphamide. A review of the literature revealed that young age may be a good prognostic sign and that immunosuppressive drugs and supportive management are essential in the treatment. Renal biopsy is helpful in the differential diagnosis of HSP-mimicking pulmonary vasculitic syndromes. Combining cyclophosphamide with glucocorticoids may improve the outcome in severe HSP cases with pulmonary haemorrhage.
过敏性紫癜（HSP）是儿童最常见的血管炎。它是一种多系统疾病，但肺出血极为罕见。我们报告一例6岁患过敏性紫癜、肺出血及严重肾脏受累的男孩。该患者对静脉注射甲泼尼龙和环磷酰胺联合治疗有反应。文献回顾显示，年轻可能是一个良好的预后指标，免疫抑制药物和支持性治疗在该病治疗中至关重要。肾活检有助于鉴别模仿HSP的肺血管炎综合征。环磷酰胺与糖皮质激素联合使用可能改善伴有肺出血的严重HSP病例的预后。
