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Published on: December 26, 2017
Corticosteroid-sparing benefit of intravenous immunoglobulin in systemic sclerosis-associated myopathy: A comparative
Benjamin Chaigne1, Simao Rodeia2, Nouria Benmostefa3
1Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM U1016, Institut Cochin, Paris; Université Paris Descartes, Sorbonne Paris Cité, Paris; CNRS UMR 8104, Paris.
Introduction:
Little is known about systemic sclerosis (SSc)-associated myopathy (SScAM) treatment. Herein we evaluated the use of intravenous immunoglobulin (IVIg) in SScAM.
Methods:
We conducted a retrospective study of patients with SScAM in the Internal medicine department of Cochin University Hospital between 1993 and 2017.
Results:
Fifty-two patients were included comprising 18 (34.6%) with limited SSc and 34 (65.4%) with diffuse SSc. SScAM occurred at a median [interquartile range (IQR)] time of 1 month [0-15] after SSc diagnosis. Thirty-four patients (65.4%) had muscle weakness, 28 (53.8%) had myalgia and 24 (46.2%) had dysphagia. Fifty patients (96.2%) had increased creatine kinase, 22/26 (84.6%) had myopathic electromyography, 10/12 (83.3%) had a high intensity signal of girdle muscles on MRI and 49/50 (98%) had abnormal muscle biopsy. Eighteen (34.6%) patients received IVIg. Severe adverse events occurred in 3/18 (16.7%) patients. When compared to patients who did not receive IVIg, patients who received IVIg had a significantly higher maximal corticosteroid (CS) dose ever, a greater decrease of CS at 3 months, and a lower CS dose at one year and at the end of follow up.
Conclusions:
This study suggests the benefit of IVIg as adjunctive therapy, with an acceptable tolerance profile, and supports its use as a CS-sparing agent, in SScAM.
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