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Polymyositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients

Department of Internal Medicine, Vall D’Hebron General Hospital, Barcelona, Spain, aselva{at}vhebron.net

M Labrador-Horrillo

Autoimmunity Research Unit, Vall D’Hebron General Hospital, Barcelona, Spain

X Muñoz-Gall

Pneumology Service, Vall D’Hebron General Hospital, Barcelona, Spain

X Martínez-Gomez

Department of Epidemiology and Statistics, Vall D’Hebron General Hospital, Barcelona, Spain

J Majó-Masferrer

Department of General Pathology, Vall D’Hebron General Hospital, Barcelona, Spain

R Solans-Laque

Department of Internal Medicine, Vall D’Hebron General Hospital, Barcelona, Spain

C P Simeon-Aznar

Department of Internal Medicine, Vall D’Hebron General Hospital, Barcelona, Spain

F Morell-Brotard

Pneumology Service, Vall D’Hebron General Hospital, Barcelona, Spain

M Vilardell-Tarrés

Department of Internal Medicine, Vall D’Hebron General Hospital, Barcelona, Spain

The objective of this study was to assess the prevalence, clinical, histological and immunological characteristics, and the long-term outcome of polymyositis-(PM) and dermatomyositis-(DM) associated lung disease, and to define subgroups of lung-associated inflammatory myopathies. This retrospective study included 81 consecutive patients diagnosed with PM/DM. Pulmonary involvement was systematically investigated in relation to clinical symptoms by chest radiography, high resolution computed tomography and pulmonary function testing. Anti-synthetase autoantibodies (ASA) were analysed by ELISAandconfirmedbyproteinandRNA immunoprecipitation methods. Statistical analyses were done with the Student t-test and Fisher exact test. Cumulative survival probabilities were estimated by the Kaplan-Meier method and Cox regression analysis. Fifty patients (61%) presented pulmonary involvement. Thirty-two (39%) had interstitial lung disease and five of them had devastating acute interstitial pneumonia with pneumomediastinum and an unfavorable prognosis. Histology showed diffuse alveolar damage in this subgroup and ASA were negative. Eighteen patients (22%) presented restrictive myopathic lung disease; in three of them respiratory muscles could not maintain ventilation. ASA were positive in 17 of the 50 patients (34%) and were significantly associated with interstitial lung disease (OR: 4.5 [95% CI: 1.3-15.3]), arthritis (OR: 6.0 [95% CI: 1.3-29.2]) and ‘mechanic hands’ (OR: 8.5 [95% CI: 1.7-41.4]); the presence of these autoantibodies did not imply worse survival prognosis. We concluded that clinical and immunological characteristics allowed the grouping of patients into different types of PM/DM lung-associated disease. Presence of ASA did not affect survival. ASA-negative patients with acute interstitial pneumonitis and pneumomediastinum had an unfavorable prognosis.

Key Words: anti-synthetase antibodies • dermatomyositis • inflammatory myopathy • interstitial lung disease • polymyositis • restrictive myopathic lung disease

Lupus, Vol. 14, No. 7, 534-542 (2005)
DOI: 10.1191/0961203305lu2158oa


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