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A Case Study And National Database Report Of Progressive Systemic Sclerosis And Associated Conditions, David Phillips, Barbara Phillips, David M. Mannino

David M. Mannino

We report the case of a 34-year-old white woman with a history of progressive systemic scleroderma (PSS) and diffuse alveolar hemorrhage (DAH) that may be either a rare complication of PSS or induced by D-penicillamine. The DAH progressed to hemoptysis and led to intubation for airway protection. The patient progressed to acute renal failure. Her chest x-ray revealed diffuse bilateral infiltrates. She developed pulmonary fibrosis with secondary pulmonary 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 …