A long-term prospective randomized controlled study of non-specific interstitial pneumonia (NSIP) treatment in scleroderma

Abstract

The association of cyclophosphamide (CYC) and prednisone (PRED) for the treatment of lung fibrosis in systemic sclerosis (SSc) was only evaluated in uncontrolled studies, although in idiopathic interstitial lung disease (ILD) this association seems to be beneficial in patients with non-specific interstitial pneumonia (NSIP). To treat SSc-ILD in a prospective open-label controlled study based on lung pattern during 12months of treatment. A 3-year analysis was also performed. Twenty-four consecutive patients with SSc and ILD were submitted to an open lung biopsy. Eighteen patients (NSIP) were randomized in two groups: CYC versus CYC + PRED during 12months. Lung function tests (diffusion lung capacity of monoxide carbone corrected for hemoglobin concentration (DLCO-Hb), forced vital capacity (FVC), total lung capacity) and Modified Rodnan Skin Score (MRSS) were performed before, after one of treatment and after 3years from the end of the treatment. Pulmonary function tests were similar in both groups on baseline. After 1year of treatment, FVC% was comparable between CYC groups (p = 0.72) and in CYC + PRED (p = 0.40). Three years after the end of treatment, FVC% values (p = 0.39 in group CYC and p = 0.61 in CYC + PRED and p = 0.22 in CYC + PRED) and DLCO-Hb (p = 0.54 in CYC and p = 0.28 in CYC + PRED) were similar compared to 1year of treatment. We observed a reduction of the MRSS in the CYC + PRED group after 1year of treatment (p = 0.02); although after 3years, MRSS values remained stable in both groups. CYC was effective to stabilize lung function parameters in NSIP lung pattern of SSc disease for 3years after the end of a 1-year therapy.