Long-term effects of immunosuppressive therapy on lung function in scleroderma patients

The study aims to analyze the effects of induction treatment with cyclophosphamide (CYC) pulse therapy followed by maintenance treatment with other mild immunosuppressive agents on lung function in scleroderma (SSc) patients. Thirty patients with SSc (mean age 52years, mean disease duration <2years) with forced vital capacity (FVC) 80% and/or diffusing capacity of carbon monoxide (DLco) 70% were included. Monthly CYC pulses were given for 6months (induction treatment), followed by 3-monthly maintenance pulses for the next 18months, and during the next 5years patients received other mild immunosupressive therapy brought by the competent rheumatologist. The efficacy was evaluated by comparing FVC% and DLco% after 6, 24, and 84months from the baseline. All patients completed induction and maintenance treatment with CYC. Three patients were lost to follow-up. The rest of 27 patients, during the next 5years, received other immunosupressive agents (14 azathioprine, 9 methotrexate, and 4 mycophenolate mofetil). Three patients died in the 4years of follow-up. By 6, 24, and 84months, the mean FVC and DLco changes were +0.47 and +2.10, +3.30 and -2.49, and +1.53 and -3.76%, respectively. These changes were not significantly different from the baseline values. CYC does not appear to result in clinically significant improvement of pulmonary function but fulfilled criteria of stable disease. Maintenance treatment with other mild immunosupressive agents preserves the benefits achieved during CYC treatment.