A case study and national database report of progressive systemic sclerosis and associated conditions

We report the case of a 34-year-old white woman with a history of progressi ve systemic scleroderma (PSS) and diffuse alveolar hemorrhage (DAH) that ma y be either a rare complication of PSS or induced by D-penicillamine. The D AH progressed to hemoptysis and led to intubation for airway protection. Th e patient progressed to acute renal failure. Her chest xray revealed diffus e bilateral infiltrates. She developed pulmonary fibrosis with secondary pu lmonary hypertension. She experienced a brief period of improvement of her respiratory status after steroid treatment. We also report a database of 21 ,442 decedents with PSS over a 15-year period from 1979 to 1994. Our report demonstrates that of over 21,000 decedents, only 0.2% had pulmonary hemorr hage or hemoptysis or both listed as a cause of death. The data also demons trate that PSS was the underlying cause of death more frequently in younger people. Age-adjusted mortality rates were higher for blacks than for white s and for women than for men.