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.
Immunosuppressive Treatments Do Not Alter Natural History of Sporadic Inclusion Body Myositis: The Paris/Oxford Study on 136 Patients.
Olivier2, Benveniste, Marguerite5, Guiguet, Freebody4, Jane, Herson2, Serge, Dubourg3, Odile, Squier4, Waney, Laforet1, Pascal
Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, Institut de Myologie, France
Assistance Publique- Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, Médecine Interne, France
Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, Neuropathologie, France
Department of Neurology, West Wing, John Radcliffe Hospital, Oxford, United Kingdom
INSERMUPMC UMR S 943, Biostatistique, Paris, France
Sporadic inclusion body myositis (sIBM) is characterized by slowly progressive, asymmetric atrophy and weakness of both proximal and distal muscles, most prominently affecting the finger and wrist flexors and quadriceps. Although drugs such as corticosteroids, methotrexate, cyclophosphamide, and IVIg may reduce the degree of inflammation on biopsy and lower serum creatine kinase levels, clear clinical improvement has not been demonstrated. The aim of the study was to evaluate whether immunosuppression alters the course of progression of sIBM.
Two cohorts of patients, one from Paris and the other from Oxford were included. The same clinical-research form was used in both centers for data collection and was completed either during a clinic visit (52%), or by extraction from previous medical records (48%).
136 patients (57% males, 61  years at onset) were included. sIBM was diagnosed on the basis of accepted clinical and pathological criteria; 97 patients (72%) were considered to have definite sIBM, 39 patients (28%) had probable sIBM. At the last visit all patients had muscle weakness (proximal MRC <= 3/5 in 47%, distal MRC <= 3/5 in 38%) and half of them reported swallowing problems. During their follow-up, 75% of patients had significant walking difficulties and 37% used a wheelchair (after a median duration from onset of 14 years). The risk of death was only influenced by older age at onset of first symptoms. 71 (52%) patients received immunosuppressive treatments (prednisone in 91.5% associated (in 64.8%) with other immunomodulatory drugs (intravenous immunoglobulins, methotrexate or azathioprine) for a median duration of 41 months). At the last assessment, patients who had been treated were more severely affected on a sIBM weakness composite index (p=0.03) and disability scales (Walton p=0.007, RMI p=0.004).
This study showed that in our population, sIBM started on average in the 6th decade of life and is then slowly progressed. It took a median of 14 years to get from first symptoms to the need to use a wheelchair, but also, that this disease is not usually the cause of death. Furthermore, it could not be excluded that conventional immunosuppressant treatment is not only ineffective but might also hasten progression. The findings of this study include important messages for the design of the future trials.
To cite this abstract, please use the following information:
Olivier, Benveniste, Marguerite, Guiguet, Freebody, Jane, Herson, Serge, Dubourg, Odile, Squier, Waney, et al; Immunosuppressive Treatments Do Not Alter Natural History of Sporadic Inclusion Body Myositis: The Paris/Oxford Study on 136 Patients. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :922