A 75-Year-Old Man with Slowly Progressive Leg and Hand Weakness

摘要: Sporadic inclusion body myositis (sIBM) is a unique type of idiopathic inflammatory myopathies (IIM). It mainly affects men over age 50 years. The disease characterized by prominent knee extensor and finger flexor weakness, normal or mildly elevated serum creatine kinase level, mixed myopathic neurogenic motor unit potentials on electromyography, association with an autoantibody against cytosolic 5′-nucleotide 1A (cN1A; NT5C1A), lack favorable response to immunosuppressive therapies. Muscle biopsy plays essential role in the diagnosis sIBM. typically shows combination primary endomysial inflammation, rimmed vacuoles, tubulofilamentous inclusions, excessive cytochrome c oxidase (COX)-deficient myofibers, diffuse upregulation class I major histocompatibility complex (MHC1). Here we present case IBM illustrate clinical pathological features.