Therapy for severe interstitial lung disease in systemic sclerosis. a retrospective study

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Abstract
Objective. This retrospective observational study attempted to determine whether any of the therapies used in the management of systemic sclerosis (SSc) patients held potential benefit for patients with interstitial lung disease. Methods. All patients with SSc who had a pulmonary function test (PFT) showing a forced vital capacity (FVC) of Results. Of 363 SSc patients who had an FVC < 70% predicted, 122 had a second PFT and fulfilled the criteria for one of the following drug groups: high–dose prednisone (n = 21), immunosuppressive other than cyclophosphamide (CYC) (n = 16), CYC (n = 14), D–penicillamine (n = 37), or no drug (n = 34). In both analyses, the CYC–treated group showed significantly more improvement in FVC than did the other groups. Patients with early disease had the greatest likelihood of responding to any drug. Conclusion. This retrospective study shows that patients treated with CYC had a significant improvement in FVC over time. Prospective controlled studies of CYC treatment in early disease are necessary to determine if it can significantly alter the natural history of interstitial lung disease.