Pulmonary hemorrhage associated with Henoch-Schönlein purpura in pediatric patients: case report and review of the literature.

Objectives: To characterize the clinical characteristics of pediatric patients with pulmonary hemorrhage and Henoch-Schonlein purpura (HSP). Methods: Presentation of a pediatric patient with pulmonary hemorrhage associated with HSP and review of relevant cases based on a PubMed search (1966 to April 2010). Results: We identified 17 previously reported pediatric cases with HSP and pulmonary hemorrhage. The most frequent clinical manifestations were rash (17 patients, 100%), proteinuria (14 patients, 82%), and abdominal pain (13 patients, 76%). Six patients (35%) had complete resolution of symptoms; 7 patients (41%) had partial recovery, and 4 patients (23%) died. Nine patients (53%) had acute respiratory failure following intubation and 3 of these patients (33%) died. Five patients were treated with methylprednisolone pulse therapy and 1 was also given low-dose cyclophosphamide treatment, but 2 of these 5 patients (40%) died. Three patients were given cyclophosphamide pulse therapy plus steroid (nonpulse or pulse) therapy, and all survived. Among the 6 nonintubated patients, all were given steroid treatment with or without an immunosuppressant drug, and all survived. In our reported case, plasma exchange appeared to help resolve the pulmonary hemorrhage and crescentic glomerulonephritis that were associated with HSP. Conclusions: For pediatric HSP patients with pulmonary hemorrhage but no respiratory failure, methylprednisolone pulse or nonpulse therapy could be the first-line therapy. In the presence of respiratory failure, cyclophosphamide pulse therapy is suggested. Plasma exchange may be considered for treatment of pulmonary renal syndrome or refractory pulmonary hemorrhage. (C) 2011 Elsevier Inc. All rights reserved. Semin Arthritis Rheum 41:305-312