Pulmonary alveolar hemorrhage in patients with rheumatic diseases in Korea- Clinical presentation, treatment, survival, and outcome

Pulmonary alveolar hemorrhage (PAH) is a rare and often fatal presenting fe ature of rheumatic diseases, with high mortality rate ranging from 40% to 9 0%. This study was undertaken to review the clinical manifestations, diseas e course, prognosis, and treatment of PAH in rheumatic diseases in Korea. A retrospective analysis was performed from October 1995 to March 1999 at th e Samsung Medical Center. Ten cases were diagnosed as having pulmonary hemo rrhage with rheumatic diseases that comprised the following: 6 systemic lup us erythematosus (SLE), 3 microscopic polyangiitis (MPA), and 1 mixed conne ctive tissue disease (MCTD). in 80% of the patients in the present series, PAH was the first clinical manifestation of rheumatic diseases. The most co nsistent systemic manifestation occurring in conjunction with PAH was renal involvement (80%). The overall patient mortality rate was 50% (5/10) in th e current series. Our study suggests that PAH often occurs as the first cli nical manifestation of rheumatic diseases and needs urgent medical treatmen t including plasmapheresis in addition to cyclophosphamide and methylpredni solone.