Rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis.

BACKGROUND: A 36-year-old woman developed new-onset Raynaud phenomenon and rapidly progressive dyspnea over a 2-week period. A lung biopsy demonstrated pauci-inflammatory nonspecific pneumonitis, which proved refractory to systemic corticosteroid and intravenous cyclophosphamide therapy. Her preterminal course in an intensive care unit was typified by sequential organ failure. Postmortem examination showed extensive organ fibrosis, including severe diffuse alveolar damage and parenchymal fibrosis, and a notable lack of potentially treatable tissue inflammation.

INVESTIGATIONS: Chest radiography, physical examination, screening for autoantibodies, measurement of serum creatinine, creatine phosphokinase, and brain natriuretic peptide levels, cardiac examination, pulmonary function tests, electrocardiography, transthoracic Doppler echocardiography, right heart catheterization, high-resolution thoracic CT, pulmonary ventilation/perfusion scan, lung biopsy.

DIAGNOSIS: Interstitial lung disease associated with diffuse systemic sclerosis.

MANAGEMENT: Treatment with oxygen, oral and intravenous corticosteroids, mycophenolate mofetil and intravenous cyclophosphamide.