ABSTRACT
Juvenile dermatomyositis (JDM) is an autoimmune disease manifesting as proximal muscle weakness and skin rash and can involve multiple systems and visceral organs. Myositis-specific autoantibodies (MSAs) are highly associated with various complications and prognosis in JDM. Patients with anti-Mi-2 antibodies tend to have good prognosis and typical clinical symptoms. Patients with anti-MDA5 antibodies often have diffuse interstitial lung disease and skin ulcer, with mild symptoms of myositis. Patients with anti-NXP2 antibodies often have calcinosis, and such antibodies are associated with gastrointestinal bleeding and perforation. Patients with anti-TIF1-γ antibodies have diffuse and refractory skin lesions. Anti-SAE antibodies are rarely detected in children, with few reports of such cases. This article reviews the features of clinical phenotypes in JDM children with these five types of MSAs, so as to provide a basis for the clinical treatment and follow-up management of children with JDM.
Subject(s)
Humans , Autoantibodies , Dermatomyositis , Lung Diseases, Interstitial/etiology , Myositis , PrognosisABSTRACT
Objective To investigate the effect of transcriptional internediary factor 1 gamma (Tif1γ)and its signal pathway related proteinson apoptosis of human pancreatic cancer cell line Capan-1 treated with either gemcitabine (GEM) or raltitrexed (RTX).Methods Capan-1 cells were treated with GEM of 559 μmol/L or RTX of 0.86 μmol/L for 36 h.The cell apoptosis of Capan-1 was determined using flow cytometry.Protein levels of Tif1γ,TGF-β1,Smad3,p-Smad3,Smad4,Bcl2,BAX and Caspase3 in Capan-1 cells were determined by Western blot.Results The late apoptosis and death ratio after RTX treatment were 28.7％ ± 5.1％ and 3.7％ ± 0.5％,respectively,showing significant difference from that after GEM treatment or untreated control group (P＜0.01).The results of Western blot showed that the relative protein levels of Tif1 γ,TGF-β1,p-Smad3,BAX and Caspase3 in Capan-1 cells were increased in RTX group compared with those in GEM group or control group (P＜0.05).The relative protein levels of Smad4 and the Bcl2/Bax ratio were decreased in RTX group compared with those in GEM group or control group (P＜0.05).Conclusions Increased level of Til1γ by RTX treatment resulted in decreased level of Smad4 to regulate the balance of Bcl2/Bax,increased Caspase3 and increased apoptosis in Capan-1 cells.
ABSTRACT
Objective To study the relationship between anti-transcription intermediary factor 1 family proteins (TIF1) autoantibodies profiles and cancer-associated dermatomyositis (CAM) and to define the diagnostic value of different subtypes of anti-TIF1 aotuantibodies for CAM.Methods The sera from 156 patients with dermatomyositis (DM),55 with polymyositis (PM),70 with systemic lupus erythematosus (SLE),60 with rheumatoid arthritis (RA),46 with primary Sj(o)gren syndrome (pSS),14 with systemic sclerosis (SSc),49 with kinds of malignancies and 40 healthy subjects were examined by immunoprecipitation assays followed by western blotting.Statistical analysis were performed using ANOVA,t test Mann-Wittney U and x2 test or Fisher exact test,nonparametric method was used to evaluate the sensitivity and specificity through calculating the area under the receiver operating characteristic curve (ROC).Results In summary,32 of 156 sera from patients with DM (20.5％) were positive for at least one anti-TIF1 autoantibodies.There are four subtypes of anti-TIF1 autoantibodies profiles existed in patients with DM,including 4 patients with only positive anti-TIF1-α (12.5％),20 with only positive anti-TIF1-γ (62.5％),7 with both positive anti-TIF1-α and anti-TIF1-γ (21.9％) and 1 with both positive anti-TIF1-β and anti-TIF1-γ (3.1％).However,only positive anti-TIF1-α (7.3％) was observed in 4 patients with PM.No patients with other CTDs as well as malignancy and healthy subjects were positive for these autoantibodies.The sensitivity and specificity of presence of anti-TIF1-α antibodies for the diagnosis of CAM were 42.9％ and 96.5％,respectively and those of anti-TIF1-β antibodies were 0 and 99.3％,respectively and those of anti-TIF-1-γ antibodies were 64.3％ and 86.6％,respectively.Application of areas of ROC to identify the best performance of test of anti-TIF1 antibodies profiles were 0.70,0.50,0.76,0.74 and 0.71,respectively.Conclusion Joint detection of antiTIF1 autoantibodies profiles can improve the diagnostic capbility for CAM.