INTERSTITIAL LUNG-DISEASE RELATED TO DERMATOMYOSITIS - COMPARATIVE-STUDY WITH PATIENTS WITHOUT LUNG INVOLVEMENT

Objective, To compare clinical and histopathological data and outcome of patients with dermatomyositis (DM) with and without interstitial lu ng disease (ILD). Methods. Patients diagnosed with definite DM were pr ospectively evaluated. Clinical and analytical data were recorded and muscle biopsies were performed. In patients with respiratory symptoms an extensive pulmonary evaluation was done. Results. 104 patients were diagnosed with idiopathic inflammatory myopathy, 63 with DM. Eight of the patients with DM (13%) also had associated ILD. Arthralgia and fe ver were more frequently seen in the ILD group and there was no associ ated malignant condition, Either atypical or nonspecific cutaneous les ions were more frequently seen in ILD patients, Anti Jo-1 antibodies w ere positive in 75% of the patients with ILD and in 3% of patients wit h DM without ILD (p < 0.001). Bronchoalveolar lavage measured for cell count showed an increased lymphocyte count in 3/5 cases in which this data was recorded. Lung biopsies showed interstitial pneumonitis in 2 cases and desquamative interstitial pneumonitis in one, All patients achieved complete remission of muscular involvement. With respect to l ung disease, 4/8 obtained complete remission, and the other 4 showed p artial improvement, No significant statistical differences in terms of survival were found between the 2 groups. Conclusion. ILD associated with DM represents a subgroup of DM with clinical and biological diffe rences, However, such patients do not have poorer prognosis than patie nts without ILD if they are managed aggressively with immunosuppressiv e drugs.