Usefulness of cyclosporine A on rapidly progressive interstitial pneumonia in dermatomyositis.

Background: Interstitial pneumonia in polymyositis and dermatomyositis (PM/DM) is recognized to be a major complication of PM/DM. Rapidly progressive interstitial pneumonia (RPIP) in DM is frequently refractory to steroids and is the prognosis-deterinining factor in DM. Recently cyclosporine A (CyA) has emerged as an available treatment for interstitial pneumonia in PM/DM, but its efficacy on RPIP is unclear. Aim of the work: To evaluate the usefulness of CyA in the treatment of RPIP in DM. Methods: We reviewed the medical charts of 10 cases with RPIP in DM to whom CyA was administered. Results and Conclusions: Combined administration of oral CyA and steroids with/without immuno-suppressants was likely to be beneficial in 4 of 10 cases that were refractory to the conventional preceding therapy. The values of PaO2/FIO2 when CyA were initiated were suggested to be a prognosis-determining factor for the outcome of the disease, showing that initiation of CyA should be considered in the early stage of RPIP in DM. The dosage of steroids was tapered in 3 out of 4 CyA-responsive cases without re-exacerbation. Therefore administration of CyA might be useful in lowering the dosage of steroids.