Clinical Analysis And Study On The Immunopathogenesis Of Dermatomyositis

Part One Clinical Analysis of 160 cases of Dermatomyositis【Objective】To investigate the clinical characteristics of dermatomyositis(DM).【Methods】The clinical manifestations and laboratory test results of 160 cases of DM hospitalized in Union Hospital were analyzed. Meanwhile, The clinical presentation and laboratory test results of 116 DM/PM patients and 33 DM/PM patients with ILD, 27 JDM and 133 ADM were analyzed contrastively.【Results】For the group with ILD, arthralgia, fever, higher values of erythrocyte sedimentation rate(ESR) and presence of anti-Jo-1 antibody were significantly more frequent. Compared with DM/PM without ILD, there was significantly less heliotrope rash in ILD. The ratio of male to female was 1:1.7, and cutaneous lesion was most common initial presentation. Compared with adult group, helitrope rash was significantly more common in children group.Fever, arthralgia, myocardial strain and dysphagia were significantly less frequent in children group than in adult group. There were no interstitial lung disease and malignant tumer in children group.【conclusion】women were more frequently affected than men, and cutaneous lesion was most common initial presentation. The most frequent cutaneous features were Heliotrope rash; Symmetrical weakness of the proximal muscles of four limbs were most. Organs involved in DM were heart, gastrointestinal tract and lung, and few renal involvement were noticed. 5% of patients with DM had malignant tumor. For the group with ILD, arthralgia, fever, higher values of erythrocyte sedimentation rate(ESR) and presence of anti-Jo-1 antibody were significantly more frequent. Our study underlines the high frequency of ILD in DM/PM patients and emphasizes the need for pulmonary screening in all DM/PM patients. Arthralgia, fever, higher values of erythrocyte sedimentation rate(ESR) and presence of anti-Jo-1 antibody may be predictive factors of ILD. Girls were more affected with JDM, and the prognosis was good in JDM because of mild systemic involvement and no malignant tumor. The mainstay of therapy is corticosteroids.Part two Significance of T Lymphocyte Subsets and Expression of the Costimulatory Molecules from Patients with Dermotamyositis【Objective】To investigate the expression and role of costimulatory molecules CD28,4-1BB and ICOS and T lymphocyte subsets in patients with Dermatomyositis befor and after treatment..【Methods】CD3,CD4,CD8,CD28,4-1BB and ICOS in peripheral blood of 24 DM patients and 14 normal persons were measured by immunofluorescent labeling technology and flow cytometry analysis.【Results】Compared with normal group,CD8+T cell subsets were signifcantiy decreased and the ratio of CD4/CD8 were increased. The expression percentage of CD28+ including CD4+CD28+ and CD8+CD28+ in T cells were reduced markerly, while the expression percentage of 4-1BB in T cells was significantly increased than that in normal control group.【Conclusion】Our study suggests that cosimulatory molecule CD28,4-1BB and abnormal T subsets may play an important role in pathogenesis of Dermotamyositis...