Objective.
Myositis-specific
autoantibodies(MSAs) are useful tools for identifying clinical subsets of patients with
idiopathic inflammatory myopathies(IIMs). There have been few reports on
antibodies to some DNA mismatch repair
enzymes (MMREs) inpatients with IIMs. This study was undertaken to determine the frequencies and clinical associations of
antibodies to 7 types of MMREs (MLH1, MLH3, MSH2,MSH3, MSH6, PMS1, and PMS2) in patients with IIMs and other systemic
autoimmune diseases.Methods. Clinical data and serum samples were collected from 239 Japanese patients with IIMs (147 with adult
dermatomyositis, 13 with
juvenile dermatomyositis,57 with
polymyositis, and 22 with
myositis overlap syndrome). One hundred patients with other diseases, including 40 with
systemic lupus erythematosus(SLE), were assessed as disease controls. The presence of anti-MMRE
antibodies in serum was examined by immunoprecipitation,
enzyme-linked immunosorbenassay, and immunoprecipitation/Western blotting.Results. Anti-MMRE
antibodies were found in 1 patients with IIMs and 3 patients with SLE. They were restricted to MLH1, PMS1, MSH2, and PMS2, with simultaneous positivity for more than one of these
antibodies occurring in some patients. Nine IIM patients with anti-MMREs also had other MSAs and their associated clinical features. All patients with anti-MMREs were still living at the time of the present analysis.Conclusion. Anti-MMRE
antibodies, which often co exist with other MSAs, may be serologic markers forgood prognosis in IIMs.