Background/Purpose: Systemic sclerosis (SSc) is a progressive fibrotic and autoimmune disease, which results to severe systemic complications. Rituximab (Rtx), an anti CD-20 antibody, has recently been started to use for complications of SSc especially interstitial lung disease (ILD). We aimed to asses the efficacy of Rtx therapy in SSc patients with ILD.

Methods: 39 SSc patients with progressive ILD disease included in to the study. 35 (89.7%) of these patients had experienced a worsening of ILD after the conventional cyclophosphamide therapy and 4 (10.2%) patients refused using cyclophosphamide because of potential infertility. All patients were treated with Rtx 1000 mg twice/six months. Functional vital capacity (FVC), diffusing capacity for carbon monoxide (DLCO), high resolution computed tomography (HRCT), modified Rodnan skin score (mRSS), Valentini activity index (VAI), Medsger disease severity index (MDSI) were recorded. Patients were eveluated with 6 months intervals for all these parameters. Initial and 12^th month values were assessed and data was analysed by SPSS 18.0 (SPSS, Chicago, IL). Paired t and Wilcoxon Signed Ranks tests were used.

Results: Women (37; 94.9%) outnumbered men (2; 5.1%) significantly. Mean age was 58.1±11.8 years. Median disease duration was 30 (18-132) months. Median follow-up time was 25 (18-100) months. Demographic data were given in table 1. All of the patients had active ILD which demonstrated by ground-glass opacification on HRCT. From 35 patients resistant to cyclophosphamide; 26 (66.6%) switched to Rtx monotherapy, 7 (17.9%) continued with Rtx+cyclophosphamide combination therapy, 2 (5.1%) continued with Rtx+mycophenolate mofetil combination therapy. Additionally, 6 (15.4%) patients received IV iloprost and 3 (7.7%) received bosentan. mRSS was 9.1±5.9, VAI was 2.7±0.8, MDSI was 4.4±1.6 before Rtx. FVC (%), DLCO (%) were 86.4±21.0 and 64.2±17.9 respectively. The 12^th month values were compared within initial ones and we demonstrated that mRSS (6.9±4.5, p<0.001), MDSI (4.0±1.2, p=0.032) scores had significant improvement. On the other hand VAI (2.4±0.5, p=0.051), FVC (92.1±17.0, p=0.056) and DLCO (68.1±16.1, p=0.271) values had no significant difference (table 2). Interestingly number of the patients with digital ulcers were decreased from 16 (41.0%) to 9 (23.0%).

Conclusion: In SSc, progression of ILD seems remained stable with Rtx therapy. This might be a good opportunity for the treatment. Skin score improvement in these patients might indicate the benefical effect of Rtx either.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-of-rituximab-in-systemic-sclerosis-with-interstitial-lung-disease/