Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


Intravenous Immunoglobulin (IVIg) in the Treatment of Systemic Sclerosis (SSc) Associated Myopathy.

Dzeing-Ella7,  Arnaud, Berezne7,  Alice, Ranque4,  Brigitte, Authier1,  François-Jérôme, Allanore5,  Yannick, Cabane3,  Jean, Hachulla2,  Eric

Service d'Histologie, Hôpital Henri-Mondor, Creteil, France
Service de Medecine Interne, Hôpital Claude-Huriez, Lille
Service de Medecine Interne, Hôpital Saint-Antoine, Paris, France
Service de Medecine Interne, HEGP, Paris, France
Service de Rhumatologie A, Hôpital Cochin, Paris, France
Universite Paris Descartes, Faculte de Medecine, Pole de Medecine Interne et Centre de Reference Pour les Vascularites Necrosantes et la Sclerodermie Systemique, Hopital Cochin, Assistance Publique-Hopitaux de Paris, Paris, France
Universite Paris Descartes, Faculte de Medecine, Pole de Medecine Interne et Centre de Reference Pour les Vascularites Necrosantes et la Sclerodermie Systemique, Hopital Cochin, Assistance Publique-Hopitaux de Paris, Paris

Background:

Skeletal muscle involvement is a common feature in systemic sclerosis (SSc). Conflicting results have been reported regarding the correlation between clinicobiological presentation and pathological muscle features, nevertheless there is a general agreement that histologically proven inflammatory myopathies usually regress under high-dose corticosteroid therapy. However, use of high dose of corticosteroids could participate to the induction of scleroderma renal crisis. IVIg are used for years in the treatment of dermatomyositis and polymyositis. In association with corticosteroids and methotrexate, IVIg could help control the SSc-associated inflammatory myopathy, allowing to avoid high dose corticosteroids.

Objective:

To evaluate the efficacy and the tolerance of IgIV in the treatment of SSc-associated inflammatory myopathy.

Patients and Methods:

Sixteen patients (14 females, 87.5%) with SSc-associated myopathy and available muscle biopsy were restrospectively investigated from the charts of four hospital centres for the efficacy of IVIg. All of them fulfilled the American College of Rheumatology and/or Leroy and Medsger criteria for the diagnosis of SSc. Inflammatory myopathy was either biopsy proven or based on We retrospectively include 16 patients. All patients fulfilled the American College of Rheumatology and/or Leroy and Medsger criteria for the diagnosis of SSc and had a history of myopathy explored by muscle biopsy. Myopathy was defined as the presence of muscle weakness, myalgia, or creatine kinase (CK) greater than 5 N (upper normal range), together with evidence of muscle involvement on electromyography (low voltage and/or short duration potential during maximal contraction, fibrillation or sharp wave) or on muscle biopsy.

Results:

The 16 patients had a mean age of 40.4 years, a diffuse SSc in 87.5% of the cases with a mean Rodnan score of 16 at the time of inclusion into the study. Patients were followed for a mean of 2 years. Ninety three percent of patients had interstitial lung disease. Fifty percent of the patients had pulmonary fibrosis and/or left ventricular failure. Anti-PM-Scl and anti-RNP autoantibodies were detected in 18.75% and 12.5% of the patients, respectively. All patients received IVIg in the setting of failure of corticosteroids and immunosuppressants. IVIg were prescribed at a dose of 2 mg/kg every 4 weeks. Fourteen (87.5%) patients had a favorable outcome, with complete response in 5 patients and partial response in others. Half of patients relapsed after a mean follow up of 6.4 months after interruption of IVIg treatment. The treatment was well tolerated and no case of renal failure was notified.

Conclusion:

IVIg may be helpful in the treatment of SSc-associated myopathy in case of failure of corticosteroids and/or methotrexate. However, the therapeutic effect is only suspensive. Prospective randomized studies are needed in order to evaluate the true efficacy of IVIg in SSc-associated myopathy.

To cite this abstract, please use the following information:
Dzeing-Ella, Arnaud, Berezne, Alice, Ranque, Brigitte, Authier, François-Jérôme, Allanore, Yannick, Cabane, Jean, et al; Intravenous Immunoglobulin (IVIg) in the Treatment of Systemic Sclerosis (SSc) Associated Myopathy. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :576
DOI: 10.1002/art.28345

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