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Autoimmun Rev. 2017 Apr;16(4):377-384. doi: 10.1016/j.autrev.2017.02.008. Epub 2017 Feb 13.

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

Author information

1
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).
2
AP-HP, Hôpital Saint Antoine, Service de Médecine Interne, Paris, France; UPMC Université Paris 06, Faculté de Médecine Pierre et Marie Curie, Paris, France.
3
Service d'Immunologie Clinique, Hôpitaux universitaires de Strasbourg, UPR CNRS 3572, Strasbourg, France.
4
Service de Médecine Interne et Maladies Multi-Organiques de l'Adulte, Hôpital Saint-Éloi, Centre Hospitalier Régional Universitaire de Montpellier, Montpellier, France.
5
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.
6
Service de Médecine Interne, Centre Hospitalo-Universitaire de Rennes, Université de Rennes 1, Rennes, France.
7
Service de Médecine Interne, Néphrologie et Médecine Vasculaire, Centre Hospitalier de Valenciennes, Valenciennes, France.
8
Service de Dermatologie, Hôpital Tenon, AP-HP, UPMC, Paris, France.
9
Service de Médecine Interne, Université Grenoble Alpes, Centre Hospitalier Universitaire (CHU) de Grenoble, Grenoble, France.
10
Service de Médecine Interne, Hôpital Jean-Verdier, AP-HP, Université Paris-13, Bondy, France.
11
Service de Médecine Interne et Rhumatologie, GH Diaconesses Croix Saint Simon, Paris, France.
12
Service de Médecine Interne et Rhumatologie 3C/5D, Centre Hospitalier Universitaire Pierre Zobda-Quitman, Fort-de-France, Martinique.
13
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.
14
Service de Médecine interne, Hôtel-Dieu, CHU de Nantes, Université de Nantes, Nantes, France.
15
CHU, Université de Toulouse, Faculté de Médecine, Service de Médecine Interne, Toulouse, France; INSERM, UMR 1027, Toulouse, France.
16
Univ. Lille, INSERM U995 - LIRIC - Lille Inflammation Research International Center, F-59000 Lille, France; INSERM, U995, F-59000 Lille, France.
17
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).
18
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.

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).

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.

KEYWORDS:

Fibrosis; Inflammatory myopathies; Intravenous immunoglobulins; Systemic sclerosis

PMID:
28232167
DOI:
10.1016/j.autrev.2017.02.008
[Indexed for MEDLINE]

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