We report 3 patients with polymyositis and the anti-Jo-1 antibody who developed fatal adult respiratory distress syndrome (ARDS). Other than the presence of the anti-Jo-1 antibody, there were no other consistent clinical features at the onset of disease that were predictive of ARDS development.The inflammatory myopathies are a heterogeneous group of idiopathic, chronic inflammatory disorders of skeletal muscle. Pulmonary involvement in myositis may be primary (interstitial lung disease; ILD) or secondary to respiratory muscle weakness or other organ involvement (e.g., pulmonary edema due to heart failure or aspiration due to the proximal dysphagia of pharyngeal involvement). Drug-induced pneumonitis or pulmonary fibrosis as well as bacterial or opportunistic infections also lead to pulmonary complications (1).ILD is commonly associated with the inflammatory myopathies (2,3). The clinical characteristics of ILD in myositis are similar to those observed in primary forms of ILD or other connective tissue diseases. A severe, rapidly progressive, and fatal diffuse alveolitis may occur, but most patients have a mild, slowly progressive form of ILD. Occasionally, patients have only asymptomatic radiographic changes, such as bibasilar fibrosis or minor abnormalities in pulmonary function testing.A unique feature of ILD in myositis is its strong association with the anti-aminoacyl-transfer RNA (tRNA) synthetase autoantibodies (4). The anti-Jo-1 antibody is the most frequently identified myositisSupported by a grant from the Commonwealth of Pennsylvania, Department of Health.Kevin Clawson, DO, Chester V. Oddis, MD: University of Pittsburgh, Pittsburgh, Pennsylvania.Address reprint requests to Chester V. Oddis, MD, Medicine/Rheumatology, University of Pittsburgh, 3471 Fifth Avenue, Suite 502, Pittsburgh, PA 15213-3221.Submitted for publication December 16, 1994; accepted in revised form April 8, 1995. specific antibody, and is the most common of the group of anti-aminoacyl-tRNA synthetases. It is found in approximately 20% of patients with myositis (9, and is itself directed against histidyl-tRNA synthetase. The onset of illness in anti-Jo-1 antibody-positive patients is often rapid, with any combination of symptoms, including fever, Raynaud's phenomenon, "mechanic's hands," polyarthritis, myositis, and ILD. The response to therapy in this subset of myositis patients has been poor; relapses are frequent and the 5-year cumulative survival is less than 70% (6).In the present report, we describe 3 patients with the anti-Jo-1 antibody who developed rapidly progressive respiratory failure and fatal adult respiratory distress syndrome (ARDS).
CASE REPORTSPatient 1. Patient 1, a 64-year-old white woman, developed fatigue, muscle weakness, myalgias, and arthritis in October 1993. One month later, she was admitted to a local hospital with fever and proximal dysphagia. The initial examination revealed a body temperature of 38.8"C, orthostatic hypotension, inspiratory rales, and moderate proximal muscle weakness. The hemogram, ery...