Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study

The aims of this study were to retrospectively review Japanese consecutive cases of polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM), focusing on interstital lung disease (ILD) and malignancy, and to document any differences in the incidence, clinical features, and impact on prognosis among patients with PM, DM, and CADM. We retrospectively reviewed 62 consecutive patients diagnosed with PM, DM, and CADM according to Bohan and Peter's criteria (PM/DM) and Sontheimer's criteria and Gerami's criteria (CADM), focusing on ILD and malignancy. ILD occurrence rates were 48 % (11/23) in patients with PM, 46 % (11/24) in DM, and 100 % (15/15) in CADM. Malignancy occurred during diagnosis or the observation period in 14 patients; 86 % were without ILD, and 64 % were DM without ILD. Multivariate logistic regression analysis showed that the risk of newly diagnosed malignancy was significantly lower in patients with ILD [odds ratio, 0.0688; 95 % confidence interval (CI), 0.00127-0.372; p = 0.00190] and significantly higher in patients with DM (odds ratio, 5.21; 95 % CI, 1.17-23.1; p = 0.0299) than in other patients. Patients with malignancies had shorter survival than those without malignancies; no clinically meaningful difference in survival was observed among the different myositis types and for presence of ILD. In CADM-ILD, 80 % fatal cases died from refractory ILD ≤90 days from the first visit; neither death nor recurrence occurred subsequently. In conclusion, a positive association between DM and malignancy and a negative association between ILD and malignancy were noted. In the present study, malignancy was a predictor of poor long-term prognosis, but ILD were not. ILD associated with CADM contributed greatly to poor short-term prognosis, but neither death nor recurrence occurred subsequently.