[Inclusion-body myositis]

Abstract

Sporadic inclusion-body myositis (sIBM) presents in average at the sixth decade of life and affects three men for one woman. It is a non-lethal, slowly progressive but disabling disease. Except the striated muscles, no other organs (such as the interstitial lung) are involved. The phenotype of this myopathy is particular since it involves the axial muscles (camptocormia, swallowing dysfunction) and limb girdle (notably the quadriceps) but also the distal muscles (in particular the fingers' and wrists' flexors) in a bilateral but non-symmetrical manner. The clinical presentation is then very suggestive of the diagnosis, which remains to be proven by a muscle biopsy. Histological features defining the diagnosis associate endomysial inflammatory infiltrates with frequent invaded fibres (the myositis) and amyloid deposits generally accompanying rimmed vacuoles (the inclusions). There is still today a debate to know if this disease is at its beginning a degenerative or an auto-immune condition. Nonetheless, usual immunosuppressive drugs (corticosteroids, azathioprine, methotrexate) or polyvalent immunoglobulines remain ineffective and even may worsen the handicap. Some controlled randomized trials will soon be launched for this condition, but for now, the best therapeutic approach to slow down the rapidity of progression of the disease is to maintain muscle exercise with the help of the physiotherapists.

Keywords: Histologie musculaire; Inclusion-body myositis; Muscle pathology; Myosite; Myosite à inclusions; Myositis; Pathophysiology; Physiopathologie; SIBM; Traitement; Treatment.