title: A case study and national database report of progressive systemic sclerosis and associated conditions.
abstract: 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 1994. Our report demonstrates that of over 21,000 decedents, only 0.2% had pulmonary hemorrhage or hemoptysis or both listed as a cause of death. The data also demonstrate that PSS was the underlying cause of death more frequently in younger people. Age-adjusted mortality rates were higher for blacks than for whites and for women than for men.
authors.affiliation: Department of Medicine, University of Kentucky College of Medicine, Lexington, USA.
authors.forename: D
authors.initials: D
authors.lastname: Phillips
authors.forename: B
authors.initials: B
authors.lastname: Phillips
authors.forename: D
authors.initials: D
authors.lastname: Mannino
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology*; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018501:Antirheumatic Agents / Q000009:adverse effects; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D006469:Hemoptysis / Q000209:etiology*; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010396:Penicillamine / Q000009:adverse effects; D011650:Pulmonary Alveoli; D011654:Pulmonary Edema / Q000209:etiology*; D011658:Pulmonary Fibrosis / Q000209:etiology*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy / Q000401:mortality*; D014481:United States / Q000453:epidemiology
chemical_list: D018501:Antirheumatic Agents; D010396:Penicillamine