INCLUSION-BODY MYOSITIS AND MYOPATHIES

Sporadic inclusion body myositis is a frequent, acquired, adult-onset vacuolar myopathy affecting proximal and distal muscles with a distinc t, easily identifiable clinical pattern. Although its primary cause is still unknown, autoimmune, viral, and degenerative processes, alone o r in combination, are being considered. A uniform and sustained therap eutic response using the currently available immunomodulatory agents h as not yet been achieved. Hereditary, inherited noninflammatory rimmed vacuolar myopathies with similar histologic features, collectively ca lled hereditary inclusion body myopathies, are being redefined with th e use of molecular genetics, The implications of the recent advances i n clinical and basic sciences are discussed in the present review.