Objective: We report the clinical and pathological features of myopathy with antibodies to the signal recognition particle (SRP) in 8 Chinese patients.Materials and Methods:the age of onset in 8 patients ranged from 41 to 77 years old.The disease started insidiously and duration from onset to visiting doctor between 7 and 44 months.The main symptoms were slowly progressive proximal muscle weakness in all limbs.6 of them was pure myopathy with severe muscle weakness and CK levels between 3450-5050IU/L.2 of them was combined myopathy associated with Interstitial lung disease, mild muscle weakness and mild elevated CK levels between 300-1250IU/L.Serum myositis antibody profiles were tested with immunoblotting in them.Muscle biopsies were performed in all patients and the specimens were examined with standard histological, enzyme histochemical and immunohistochemical methods.The first antibodies in the immunohistochemical staining were mouse anti human monoclonal antibodies inlcuding CD8 for T-lymphocytes, CD20 for B-lymphocytes, CD68 for macrophages, MHC-1 for muscle memrbrane.Results: 8 cases showed stark positive of anti SRP antibody, 3 of them with positive of anti Ro-52 antibody.The muscle biopsies in 6 pure myopathy patients showed necrotic and regenerative muscle fibers associated with infiltrates of macrophage in them, but minimal T or B-lymphocytes in 2 patients.2 of them presented with fiber hypertrophy and proliferation of connective tissue.2 combined myopathy presented with only a few regenerative fibers.Conclusions: myopathy with SRP have pure myopathy with severe muscle lesions and a combined myopathy with mild muscle lesions.All of them should be considered as an antibody related macrophage myostis characterized by muscle fiber necrosis and regeneration.