Efficacy of Combined Therapy with Cyclosporin and Low-Dose Prednisolone in Interstitial Pneumonia Associated with Connective Tissue Disease

Background: The optimal treatment method for interstitial pneumonia (IP) with connective tissue disease (CTD) remains controversial. In addition, a clinically meaningful end point to judge drug efficacy has not been fully investigated. Objectives: The aim of this study was to evaluate, from various aspects, the therapeutic benefit and tolerability of combined therapy with cyclosporin A (CsA) and low-dose prednisolone (PSL) for chronic fibrosing CTD-IP patients. Methods: A total of 26 CTD-IP patients diagnosed by surgical lung biopsy and subsequently treated with the above combination therapy were retrospectively reviewed. The therapeutic regimen comprised methylprednisolone induction therapy for 2-4 weeks, followed by a combined therapy of CsA with low-dose PSL for 1 year. Evaluation of the therapeutic benefit was based on not only pulmonary function but also exercise capacity, health-related quality of life and dyspnea. Results: After 1 year of therapy, clinically significant improvements in forced vital capacity (>= 10%), carbon monox-ide diffusing capacity of the lung ( >= 15%), 6-min walk distance ( >= 28 m), and St. George's Respiratory Questionnaire ( <= -7) were observed in 61.5, 69.2, 61.5 and 69.2% of the patients, respectively. All measurements showed statistically significant improvements compared with baseline values. The 1-year treatment did not need to be discontinued in any patients due to unacceptable toxicity, and no deaths occurred. Conclusions: Combined therapy with CsA and lowdose PSL for CTD-IP patients was well-tolerated and patients displayed a noteworthy response. (C) 2014 S. Karger AG, Basel