Abstract
Background: Interstitial pneumonia associated with clinical amyopathic dermatomyositis (CADM-IP) in Asia has been reported to progress rapidly and have a poor prognosis. Recently, intensive immunosuppressive therapy has been shown to improve the prognosis. But, the response to the treatment is still unclear due to its rarity and heterogeneity.
Aim and Methods: The purpose of this study was to characterize the patients with CADM-IP. 13 patients diagnosed with CADM-IP at our hospital from 1999 to 2014 were retrospectively reviewed. We compared the clinical features; physical background, serum markers, high-resolution computed tomography (HRCT) findings, and treatment between two groups; the survivors and non-survivors.
Results: The mean age of the patients was 50 years old; women was 61.5 %. The mortality rate was 30.7% and the patients died mainly from the progression of IP within two months after the treatment. The HRCT findings on admission were not significantly different. All the patients were treated with high-dose corticosteroids, cyclosporine (Cs) and cyclophosphamide (Cy) in the initial treatment. In the survivor group, Cs was switched to tacrolimus in the cases resistant to this treatment. In 7 cases, intravenous immunoglobulin infusions (IVIG) were administered for the progression of IP. The initiation of IVIG therapy in the non-survivor group was slightly later than in the survivor group.
Conclusions: Differences in the clinical features on admission between the two groups were not observed, whereas the secondary treatment after the failure of the initial treatment differed between the two groups. It may be important factors for improving the prognosis of CADM-IP.
- Copyright ©ERS 2015
