Combination therapy with rituximab and mycophenolate mofetil in systemic sclerosis. A single-centre case series study.

Objective. To describe a single centre experience using combination therapy with rituximab (RTX) and mycophenolate mofetil (MMF) in a prospective series of systemic sclerosis (SSc) patients with pulmonary and cutaneous involvement, rapidly progressive or resistant to conventional therapy. Methods. RTX was administered in two different regimens (1000 mg fortnightly x 2 or 375 mg/m(2)/week for 4 consecutive weeks) at baseline and after 6 months, associated with MMF 2000 mg/ day continuously. Cutaneous fibrosis was evaluated assessing modified Rodnan Skin Score (mRSS) and pulmonary involvement was evaluated performing pulmonary function tests, diffusing lung capacity for carbon monoxide and chest high-resolution computed tomography (HRCT). The radiological extension of the interstitial lung disease (ILD) at HRCT, was assessed with the conventional visual reader-based score (CoVR) and with a computerised-aided method (CaM) using a DICOM software. Results. Eighteen SSc patients underwent combination therapy (F/M: 10/8, median age 51 years, median duration of disease 27 months). Data from fifteen patients were available at 12-month follow-up. The mRSS showed a significant improvement; a significant increase in forced vital capacity and forced expiratory volume in the first second were also observed. In addition, a significant reduction of the extension of ILD was detected when evaluated with CaM. No serious adverse events were observed during the follow-up period. Conclusion. Despite preliminary results and limited to a small number of patients, our data suggest that therapy with RTX and MMF is well tolerated, safe, and potentially effective for cutaneous and pulmonary involvement in SSc.