Familial Inclusion Body Myositis (FIBM): Update

Abstract

Familial inclusion body myositis (FIBM) is extremely rare. The disease is characterized by relatively late onset, selective and early involvement of quadriceps, forearm and finger flexors, only mild increase of serum creatine kinase CK level, frequent rimmed vacuoles in muscle histopathology with substantial inflammatory cell infiltration. The combination of clinical, histological, immunopathological and immunogenetic features indicates that these patients have a disease identical to sporadic inclusion body myositis.