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

Pulmonary hemorrhage associated with Henoch-Schöenlein purpura in an adult patient primary diagnosed of IgA nephropathy

“…Intravenous pulse methylprednisolone followed by oral prednisolone (1 mg/kg/day) and a monthly intravenous cyclophosphamide pulse resolved the pulmonary hemorrhage. In a review of 36 HSP patients complicated with pulmonary hemorrhage, 27.8 % of the patients perished [Rajagopala et al,1]. While the most efficient therapeutic strategies for these patients have yet to be determined, we speculate that an aggressive therapy of pulse methylprednisolone combined with immunosuppression agents is likely to bring about the best outcome in cases with pathological conditions similar to our patient's.…”

“…Skin biopsy revealed leukocytoclastic vasculitis, a condition that supported the diagnosis of HSP as the cause of her pulmonary hemorrhage. Pulmonary hemorrhage is extremely rare as a complication of HSP: according to the literature: only 36 cases have been reported [1]. HSP is usually a self-limited condition that lasts about 4 weeks [3,4].…”

“…Rajagopala et al [1] reviewed 36 cases of HSP complicated with pulmonary hemorrhage, the only known cases reported in the English-language literature. Among those patients, 50 % were older than 20 years at presentation, 94.4 % manifested renal vasculitis, and as many as 27.8 % died [1].…”

“…Among those patients, 50 % were older than 20 years at presentation, 94.4 % manifested renal vasculitis, and as many as 27.8 % died [1]. Apart from the HSP patients with pulmonary hemorrhage, former studies have shown HSP to be a predominantly pediatric disease affecting children between the ages of 4 and 7 years (prevalence of 70 per 100,000 in that age range) [4].…”

“…The clinical manifestations and epidemiology of IgAN and HSP differ in some ways, but they are thought to be different clinical manifestations of the same disease as they share identical pathological and biological abnormalities [2]. No established treatment for pulmonary hemorrhage in HSP has been described in the literature, while patients receiving pulse methylprednisolone in combination with an immunosuppressive agent had lower mortality than steroid therapy alone in the review by Rajagopala et al [1]. Thus, pulse methylprednisolone and an immunosuppressive agent may be the best regimen available for pulmonary hemorrhage associated with HSP, but further studies are required to define the optimal clinical strategy.…”