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

Abstract

We describe a case of IgA nephropathy (IgAN) followed by pulmonary hemorrhage associated with Henoch-Schöenlein purpura (HSP) in an adult female. The patient had a history of renal insufficiency and persistent hematuria and proteinuria, without any extra-renal involvement. She was diagnosed with IgAN 7years before the onset of HSP and had received immunosuppressive therapy for 6years. One year after discontinuing oral prednisolone and mizoribine, she suffered a pulmonary hemorrhage. She presented with exacerbated urinary findings, and palpable purpura, resulting in the diagnosis of HSP. Intravenous pulse methylprednisolone followed by oral prednisolone (1mg/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., Semin Arthritis Rheum 42:391-400, 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. On the other hand, discontinuance of immunosuppressive therapy might have resulted in the aggravation of the disease, hence we should examine patients carefully not to miss the cue.