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Background  Major histocompatibility complex ( MHC ) I and  II  expression is not normally detected on sarcolemma, but is detected with lymphocytic infiltrates in immune‐mediated myositis ( IMM ) of humans and dogs and in dysferlin‐deficient muscular dystrophy.    Hypothesis/Objectives  To determine if sarcolemmal  MHC  is expressed in active  IMM  in horses, if  MHC  expression is associated with lymphocytic subtype, and if dysferlin is expressed in  IMM .    Animals  Twenty‐one  IMM  horses of Quarter Horse‐related breeds, 3 healthy and 6 disease controls (3 pasture myopathy, 3 amylase‐resistant polysaccharide storage myopathy [ PSSM ]).    Methods  Immunohistochemical staining for  MHC  I,  II , and  CD 4+,  CD 8+,  CD 20+ lymphocytes was performed on archived muscle of  IMM  and control horses. Scores were given for  MHC  I,  II , and lymphocytic subtypes. Immunofluorescent staining for dysferlin, dystrophin, and a‐sarcoglycan was performed.    Results  Sarcolemmal  MHC  I and  II  expression was detected in 17/21 and 15/21 of  IMM  horses, respectively, and in specific fibers of  PSSM  horses, but not healthy or pasture myopathy controls. The  CD 4+,  CD 8+, and  CD 20+ cells were present in 20/21  IMM  muscles with  CD 4+ predominance in 10/21 and  CD 8+ predominance in 6/21 of  IMM  horses. Dysferlin, dystrophin, and a‐sarcoglycan staining were similar in  IMM  and control muscles.    Conclusions and clinical importance  Deficiencies of dysferlin, dystrophin, and a‐sarcoglycan are not associated with  IMM . Sarcolemmal  MHC  I and  II  expression in a proportion of myofibers of  IMM  horses in conjunction with lymphocytic infiltration supports an immune‐mediated etiology for  IMM . The  MHC  expression also occured in specific myofibers in  PSSM  horses in the absence of lymphocytic infiltrates.

Major Histocompatibility Complex I and II Expression and Lymphocytic Subtypes in Muscle of Horses with Immune‐Mediated Myositis