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Rheumatology 2001; 40: 216-220
© 2001 British Society for Rheumatology

Paediatric Rheumatology

Pulmonary involvement in juvenile dermatomyositis: a two-year longitudinal study

Paediatric Rheumatology/Series Editor: P. Woo

S. Trapani, G. Camiciottoli1, A. Vierucci, M. Pistolesi1 and F. Falcini

Department of Paediatrics, Rheumatology Unit and
1 Department of Critical Care, Respiratory Unit, University of Florence, Italy

Abstract

Objective. To investigate the prevalence and features of asymptomatic pulmonary involvement in juvenile dermatomyositis (JDM).

Methods. Twelve JDM patients underwent pulmonary function tests at baseline, 12 and 24 months. Disease activity, duration, serum lactate dehydrogenase (LDH) values and antinuclear antibody (ANA) titres were also evaluated.

Results. Five patients showed lung impairment at baseline and four at 12 and 24 months. Forced expiratory volume in 1s, forced vital capacity (FVC), carbon monoxide diffusing capacity (DLCO) and alveolar volume were the most frequently altered variables, indicating a restrictive pattern and impairment of diffusion. The prevalence and features of pulmonary alterations did not change during follow-up. FVC values were significantly lower in active JDM patients and were inversely related to LDH. DLCO values were significantly lower in ANA-positive patients. About half of the patients of this small case series of JDM had asymptomatic lung disease.

Conclusions. We suggest that lung function should be evaluated at disease onset and regularly during follow-up, as pulmonary function tests can detect otherwise unpredictable pulmonary involvement.

KEY WORDS: Juvenile dermatomyositis, Pulmonary function tests.

Notes

Correspondence to: S. Trapani, Department of Paediatrics, Rheumatology Unit, A. Meyer Hospital, Via Luca Giordano 13, 50132 Florence, Italy


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