title: Intravenous immunoglobulins in systemic sclerosis: Data from a French nationwide cohort of 46 patients and review of the literature.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; INSERM, U995, F-59000 Lille, France; CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France; Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), F-59000 Lille, France; Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET).
authors.forename: Sébastien
authors.initials: S
authors.lastname: Sanges
authors.affiliation: AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France; UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Rivière
authors.affiliation: AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France; UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France.
authors.forename: Arsène
authors.initials: A
authors.lastname: Mekinian
authors.affiliation: Service d'Immunologie Clinique, Hôpitaux universitaires de Strasbourg, UPR CNRS 3572, Strasbourg, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Martin
authors.affiliation: Service de Médecine Interne et Maladies Multi-Organiques de l'Adulte, Hôpital Saint-Éloi, Centre Hospitalier Régional Universitaire de Montpellier, Montpellier, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Le Quellec
authors.affiliation: Hôpitaux Universitaires de Strasbourg, CHU Hautepierre, Service de Rhumatologie, Centre de Référence des Maladies Auto-Immunes Systémiques Rares, Université de Strasbourg, Fédération de Médecine Translationnelle de Strasbourg, INSERM UMR 1109, Strasbourg, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Chatelus
authors.affiliation: Service de Médecine Interne, Centre Hospitalo-Universitaire de Rennes, Université de Rennes 1, Rennes, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Lescoat
authors.affiliation: Service de Médecine Interne, Centre Hospitalo-Universitaire de Rennes, Université de Rennes 1, Rennes, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Jego
authors.affiliation: Service de Médecine Interne, Centre Hospitalo-Universitaire de Rennes, Université de Rennes 1, Rennes, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Cazalets
authors.affiliation: Service de Médecine Interne, Néphrologie et Médecine Vasculaire, Centre Hospitalier de Valenciennes, Valenciennes, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Quéméneur
authors.affiliation: Service de Médecine Interne, Néphrologie et Médecine Vasculaire, Centre Hospitalier de Valenciennes, Valenciennes, France.
authors.forename: Noémie
authors.initials: N
authors.lastname: Le Gouellec
authors.affiliation: Service de Dermatologie, Hôpital Tenon, AP-HP, UPMC, Paris, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Senet
authors.affiliation: Service de Dermatologie, Hôpital Tenon, AP-HP, UPMC, Paris, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Francès
authors.affiliation: Service de Médecine Interne, Université Grenoble Alpes, Centre Hospitalier Universitaire (CHU) de Grenoble, Grenoble, France.
authors.forename: Alban
authors.initials: A
authors.lastname: Deroux
authors.affiliation: Service de Médecine Interne, Université Grenoble Alpes, Centre Hospitalier Universitaire (CHU) de Grenoble, Grenoble, France.
authors.forename: Bernard
authors.initials: B
authors.lastname: Imbert
authors.affiliation: AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France; UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Fain
authors.affiliation: Service de Médecine Interne, Hôpital Jean-Verdier, AP-HP, Université Paris-13, Bondy, France.
authors.forename: Latifatou
authors.initials: L
authors.lastname: Boukari
authors.affiliation: Service de Médecine Interne et Rhumatologie, GH Diaconesses Croix Saint Simon, Paris, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Sené
authors.affiliation: Service de Médecine Interne et Rhumatologie 3C/5D, Centre Hospitalier Universitaire Pierre Zobda-Quitman, Fort-de-France, Martinique.
authors.forename: Christophe
authors.initials: C
authors.lastname: Deligny
authors.affiliation: Service de Médecine Interne 2, Centre de Référence National pour le Lupus et le Syndrome des Antiphospholipides, institut E3M, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France; Sorbonne universités, UPMC université Paris 06, 75013 Paris, France.
authors.forename: Alexis
authors.initials: A
authors.lastname: Mathian
authors.affiliation: Service de Médecine interne, Hôtel-Dieu, CHU de Nantes, Université de Nantes, Nantes, France.
authors.forename: Christian
authors.initials: C
authors.lastname: Agard
authors.affiliation: CHU, Université de Toulouse, Faculté de Médecine, Service de Médecine Interne, Toulouse, France; INSERM, UMR 1027, Toulouse, France.
authors.forename: Grégory
authors.initials: G
authors.lastname: Pugnet
authors.affiliation: Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; INSERM, U995, F-59000 Lille, France.
authors.forename: Silvia
authors.initials: S
authors.lastname: Speca
authors.affiliation: Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; INSERM, U995, F-59000 Lille, France.
authors.forename: Sylvain
authors.initials: S
authors.lastname: Dubucquoi
authors.affiliation: Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France; Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), F-59000 Lille, France; Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET).
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Hatron
authors.affiliation: Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; INSERM, U995, F-59000 Lille, France; CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France; Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), F-59000 Lille, France; Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET).
authors.forename: Éric
authors.initials: É
authors.lastname: Hachulla
authors.affiliation: Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; INSERM, U995, F-59000 Lille, France; CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France; Centre National de Référence Maladies Systémiques et Auto-immunes Rares (Sclérodermie Systémique), F-59000 Lille, France; Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET). Electronic address: david.launay@univ-lille2.fr.
authors.forename: David
authors.initials: D
authors.lastname: Launay
mesh_terms: D015331:Cohort Studies; D005260:Female; D005602:France; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000494:pharmacology / Q000627:therapeutic use*; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous
keywords: Fibrosis; Inflammatory myopathies; Intravenous immunoglobulins; Systemic sclerosis