TREATMENT OF HEMORRHAGIC LUPUS PNEUMONITIS WITH PLASMAPHERESIS

Pulmonary hemorrhage is a rare and often fatal complication of systemi c lupus erythematosus (SLE). Treatment with high-dose steroids and cyc lophosphamide has been of only modest value, with a reported mortality of up to 92%. We have recently seen three patients with active SLE wh o developed acute life-threatening pulmonary hemorrhage. Diagnostic ev aluation of these patients showed negative sputum and blood cultures, negative glomerular basement membrane antibodies, and negative antineu trophilic cytoplasmic antibodies. In two patients, an open-lung biopsy was performed, and histological examination showed granular alveolar immunofluorescence staining for immunoglobulin and complement componen ts. Treatment with plasmapheresis was initiated with prompt resolution of symptoms and clearing of chest radiograph. Two patients had recurr ent bleeds despite treatment with cyclophosphamide and high-dose stero ids and required repeated intubation. Plasmapheresis resulted in rapid radiographic and clinical improvement on each occasion. Two patients survived long-term and are presently without pulmonary problems; one p atient died of sepsis after initial response to plasmapheresis. The dr amatic improvement of the pulmonary disease in these patients leads us to conclude that rapid initiation of plasmapheresis should be strongl y considered in SLE patients with severe, life-threatening pulmonary h emorrhage. Copyright (C) 1994 by W.B. Saunders Company