Clinical, Pathological And Immunological Research Of Immune Necrotizing Myopathy

Object1. To investigate the clinical and pathological features of immune necrotizingmyopathy2. To investigate the immune pathogenesis of immune necrotizing myopathyMethods1. Statistically analyze and describe the general clinical information of62patientsdiagnosed as immune necrotizing myopathy by muscle biopsy and myopathology inour hospital from Jan,2007to Mar,2013.2. Observe the morphology of muscle fibers, organizational structure and theexpression of enzyme or mitochondria in muscle fibers by using histochemistry andenzyme histochemistry staining methods.3. Observe the expression and relationship of anti-signal recognition particle antibodywith the lymphocyte subgroup surface molecules by using immunochemistrystaining methods.Results1. Immune necrotizing myopathy patients usually had age at onset about50years old,and had no characteristic of seasonal onset. There was no gender difference and nomultiple-organ involvement.2. The disease developing speed of immune necrotizing myopathy was variable as wellas the severity of this disease. It manifested as myasthenia, amyotrophy and myalgia.There could be concomitant rashes. Proximal muscles weakness of limbs was mostobserved but neck muscles and bulbar muscles could also be involved. CK could benormal or predominant elevated, but EMGs were mostly myogenic changes.3. The myopathology of immune necrotizing myopathy was morphologically variousand overlapping patchy necrosis or widely distributed necrosis of muscle fibers wasobserved, which were vacuoles, historrhexis, coagulation, histolysis and hyalinenecrosis. It could be accompanied with plenty of phagocytosis and musclehypertrophy and hyperplasia. A few fibers were observed inflammation cellsinfiltrated. 4. In muscles of immune necrotizing myopathy,71.43%of anti-SRP19antibody,100%of anti-CD4, anti-CD68,60.71%of anti-CD8,39.29%of anti-CD20and64.29%ofanti-CD21immunochemistry staining were positive; In the muscles of anti-SRPantibody positive patients with necrotizing myopathy, the expressions of CD20andCD21were respectively55%and80%. There was statistical difference between thegroups with anti-SRP antibody positive/negative expression in muscles.Conclusion1. The clinical manifestations of immune necrotizing myopathy are not specific, butmyopathology has specific changes.2. Demonstrate immune factors involved in the pathogenesis of immune necrotizingmyopathy. In another word, anti-SRP19antibody, CD4, CD8, CD20, CD21, CD68were involved in this specific and complicated progress of this disease.