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

Sébastien Sanges (1, 2) , Sébastien Rivière (3, 4) , Arsene Mekinian (3, 4) , Thierry Martin (5, 6) , Alain Le Quellec (7) , Emmanuel Chatelus (8, 9) , Alain Lescoat (10) , Patrick Jégo (10) , Claire Cazalets (10) , Thomas Quemeneur (11) , Noémie Le Gouellec (11) , Patricia Senet (12) , Camille Francès (12) , Alban Deroux (13) , Bernard Imbert (13) , Olivier Fain (3, 4) , Latifatou Boukari (14) , Thomas Sené (15) , Christophe Deligny (16) , Alexis Mathian (17, 4) , Christian Agard (18) , Gregory Pugnet (19, 20) , Silvia Speca (1) , Sylvain Dubucquoi (1) , Pierre-Yves Hatron (1, 2) , Eric Hachulla (1, 2) , David Launay (1, 2)

1 LIRIC - Lille Inflammation Research International Center - U 995
2 Service de médecine interne [Lille]
3 CHU Saint-Antoine [AP-HP]
4 UPMC - Université Pierre et Marie Curie - Paris 6 - UFR de Médecine Pierre et Marie Curie
5 CHU Strasbourg - Centre Hospitalier Universitaire [Strasbourg]
6 ICT - Immunopathologie et chimie thérapeutique
7 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
8 Service de rhumatologie [Strasbourg]
9 IRM - Immuno-Rhumatologie Moléculaire
10 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
11 Centre hospitalier [Valenciennes, Nord]
12 CHU Tenon [AP-HP]
13 CHUGA - Centre Hospitalier Universitaire [CHU Grenoble]
14 Hôpital Jean Verdier [AP-HP]
15 Groupe Hospitalier Diaconesses Croix Saint-Simon
16 Hôpital Pierre Zobda-Quitman [CHU de la Martinique]
17 CHU Pitié-Salpêtrière [AP-HP]
18 Service de médecine interne [Nantes]
19 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
20 LEASP - Epidémiologie et analyses en santé publique : risques, maladies chroniques et handicaps

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 >1g/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±1552UI vs. 288±449UI, p<0.0001) and CRP levels (13.1±17.6mg/L vs. 9.2±16.6mg/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±11.6mg/day vs. 8.9±10.4mg/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 IVIG are a safe therapeutic option that may be effective in improving musculoskeletal involvement, systemic inflammation, digestive tract symptoms and could be corticosteroid sparing.

Mots clés: Fibrosis; Inflammatory myopathies; Intravenous immunoglobulins; Systemic sclerosis

Domaines: Immunologie

Sébastien Sanges, Sébastien Rivière, Arsene Mekinian, Thierry Martin, Alain Le Quellec, et al.. Intravenous immunoglobulins in systemic sclerosis: Data from a French nationwide cohort of 46 patients and review of the literature. Autoimmunity Reviews, 2017, 16 (4), pp.377 - 384. ⟨10.1016/j.autrev.2017.02.008⟩. ⟨hal-01781490⟩