Intravenous immunoglobulins in systemic sclerosis: Data from a French nationwide cohort of 46 patients and review of the literature

Background: As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature. Methods: 46 patients from 19 French centers were retrospectively recruited. They were included if they had a diagnosis of SSc and received at least 1 IVIG infusion at a dosage >1 g/kg/cycle. Relevant data collected at IVIG discontinuation were compared to those collected at IVIG initiation. A comprehensive literature review was performed. Results: We observed a significant improvement of muscle pain (74% vs. 20%, p < 0.0001), muscle weakness (45% vs. 21%, p = 0.01), joint pain (44% vs. 19%, p = 0.02), CK levels (1069 +/- 1552 UI vs. 288 +/- 449 UI, p < 0.0001) and CRP levels (13.1 +/- 17.6 mg/L vs. 9.2 +/- 16.6 mg/L, p = 0.001). We also noted a trend for an improvement of gastro-esophageal reflux disease (68% vs. 53%, p = 0.06) and bowel symptoms (42% vs. 27%, p = 0.06). Skin and cardiorespiratory involvements remained stable. Finally, corticosteroid daily dose was significantly lower by the end of treatment (13.0 +/- 11.6 mg/day vs. 8.9 +/- 10.4 mg/day, p = 0.01). Only two severe adverse events were reported (one case of deep vein thrombosis and one case of diffuse edematous syndrome). Conclusion: Our work suggests that WIG are a safe therapeutic option that may be effective in improving musculoskeletal involvement, systemic inflammation, digestive tract symptoms and could be corticosteroid sparing. (C) 2017 Elsevier B.V. All rights reserved.