Pulmonary Hemorrhage in Henoch-Schönlein Purpura: Case Report and Systematic Review of the English Literature

BackgroundDiffuse alveolar hemorrhage (DAH) is a rare complication of Henoch-Schönlein purpura (HSP) and data on its prevalence, management, and outcomes are scant.ObjectivesTo enable evidence-based management of DAH in HSP.MethodsA case report and a systematic review were conducted of all reported cases of DAH complicating HSP in the English literature.ResultsDAH predominantly affects older male children and adults with HSP. The occurrence of DAH in HSP is rare and the reported prevalence ranged from 0.8% to 5%. DAH occurred variably after the diagnosis of HSP, ranging from 2 days to 18 years. Hemoptysis (75%), drop in hemoglobin (74%), and chest infiltrates (94%) were the most common clinical findings. Lung biopsy showed leukocytoclastic vasculitis with alveolar hemorrhage (69.2%) or only alveolar hemorrhage (31.8%) with variable IgA staining by immunofluorescence. DAH was frequently severe and 50% of the patients required mechanical ventilation. Cyclophosphamide and pulse methylprednisolone for DAH was associated with better outcomes, particularly in patients who were already receiving steroids at the time of DAH. Steroids and immunosuppressants were administered for a median duration of 9 and 4.5 months, respectively. Systemic recurrences (27.7%) and recurrences of DAH (8.3%) were frequent. DAH was associated with high mortality (27.6%) and morbidity (persistent urinary abnormalities, 12%; chronic renal failure, 9%; complications of therapy, 27%).ConclusionsDAH is a life-threatening complication in HSP. Current protocols use pulse methylprednisolone and cyclophosphamide for 6 months.