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

•IVIG therapy is a safe therapeutic option in SSc patients.•IVIG may improve muscle, joint and digestive tract involvements in SSc patients.•IVIG may facilitate corticosteroid tapering in SSc patients.

BackgroundAs 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.Methods46 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.ResultsWe 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).ConclusionOur work suggests that IVIG are a safe therapeutic option that may be effective in improving musculoskeletal involvement, systemic inflammation, digestive tract symptoms and could be corticosteroid sparing.