Clinical analysis of elderly patients with anti-MDA5 antibody-positive dermatomyositis / 中华老年医学杂志

Objective:To retrospectively analyze the clinical characteristics of elderly patients with anti-MDA5 antibody-positive dermatomyositis.Methods:Data of 62 patients with anti-MDA5 antibody-positive dermatomyositis admitted to Second Xiangya Hospital from May 2016 to December 2019 were collected and patients were divided into an elderly group(≥60 years old, 17 cases)and a non-elderly group(<60 years old, 45 cases). The clinical manifestations, laboratory test resuls, treatment and prognosis of the patients in both groups were statistically analyzed.Results:A total of 62 patients with anti-MDA5 antibody-positive dermatomyositis were included in this study, including 17 elderly patients(27.4%)with an average age of(65.5±5.3)years and 45 non-elderly patients(72.6%)with an average age of(46.5±8.4)years.Compared with non-elderly patients, older patients had a shorter disease duration[(1.6±1.0)months vs.(3.7±3.3)months, t=3.883, P<0.001], a higher proportion of patients with exertional dyspnea(15/17 or 88.2% vs.26/45 or 57.8%, χ2=5.11, P=0.024)and with combined positive anti-Ro-52 antibodies(15/17 or 88.2% vs.26/45 or 57.8%, χ2=5.11, P=0.024), and a higher mortality rate[(12/17 or 70.6%) vs.(8/45 or 17.8%, χ2=15.748, P<0.001)]. In contrast, fewer elderly patients than non-elderly patients had the Heliotrope's sign(9/17 or 41.2% vs.38/45 or 57.8%), χ2=5.07, P=0.024). Conclusions:Elderly patients with anti-MDA5 antibody-positive dermatomyositis have a unique clinical phenotype with an acute onset, atypical rashes, severe pulmonary lesions, making treatment difficult, and have a poor prognosis.

Recent research on myositis-specific autoantibodies in juvenile dermatomyositis / 中国当代儿科杂志

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.

Progress in diagnosis and treatment of anti-MDA5 antibody-related dermatomyositis / 中华检验医学杂志

Dermatomyositis (DM) with positive anti-melanoma differentiation-associated gene 5 (MDA5) antibodies (MDA5+DM) is a kind of occasional and rare autoimmune disease. Due to the fact that MDA5+DM patients are prone to suffer from the rapid progressive interstitial lung disease (RP-ILD), and the mortality rate is extremely high (all-cause mortality at 6 months is almost 50%). In addition to lung disease, patients with MDA5+DM also suffering from the skin and muscle symptoms. The biomarkers represented by the anti-MDA5 antibody titer, ferritin, KL-6 level and CD4 +/CXCR4 +T cell percentage are considered to relate with MDA5+DM-ILD′s severity, activity evaluation, therapeutic effect monitoring, and prognosis prediction. The current therapeutic strategies for the disease is mainly combined with immunosuppression. This work systemly summarizes the diagnosis and treatment progress of anti-mda5 antibody-related dermatomyositis, which not only contributes to the research work of related disciplines, but also provides reference for clinical diagnosis and treatment.

Anti-MDA5 antibody is associated with acute/subacute interstitial pneumonia and predicts poor prognosis for interstitial lung diseases in patients with dermatomyositis / 中华风湿病学杂志

ObjectiveTo determine the serum MDA5 levels and their clinical associations in patients with polymyositis/dermatomyositis (PM/DM).MethodsSerum anti-MDA5 antibody was detected by ELISA in 119 adult PM/DM patients,30 patients with systemic lupus erythematosus(SLE),30 patients with rheumatoid arthritis (RA),15 patients with primary Sj(O)gren's syndrome (SS),21 patients with pulmonary infection and 50 healthy controls.t-test,Mann-Whitney U test or chi-square test or Fisher exact test as well as Logistic multivariate regression analysis were carried out to compare the results of this study.ResultsSerum antiMDA5 antibody positive rate in DM patients(22.6％) were significantly higher compared with that of patients with PM (0,P＜0.01),patients with SLE (3.3％,X2=5.68,P＜0.05),RA (3.3％,X2=5.68,P＜0.05),pSS (0,P＜0.05) and pulmonary infection(0,P＜0.05) and healthy controls (0,P＜0.01).In the DM subgroup,CADM patients presented a higher positive anti-MDA5 antibody rate than classic DM patients.The incidence of arthritis,fever,vrash raised CEA and CA153 level,and serum concentration of GGT and ferritin were significantly higher in the anti-MDA5 positive DM group than anti-MDA5 negative DM group (X2=4.08,8.06,6.357,32.4,4.867; Z=-2.86,-2.44; P value of all these tests were less than 0.05 ),while the rate of serum positive ANA,serum level of CK and T,NK cell counts in the peripheral blood were much lower than those in anti-MDA5 negative DM group(X2=4.08; Z=-2.072,-2.013,-2.907; all P＜0.05).Moreover,the incidence of acute/subacute interstitial pneumonia(A/SIP) was significantly higher in anti-MDA5 positive DM patients than anti-MDA5 negative DM patients.The sensitivity and specificity of anti-MDA5 antibody for diagnosing A/SIP in DM patients were 88.2％ and 94％ respectively.Additionally,logistic multivariate analysis showed that anti-MDA5 was an independent risk factor for death of interstitial lung diseases (ILD) in DM (OR=8.46,95％CI 1.77～40.36,P＜0.01).ConclusionIn Chinese PM/DM patients,serum anti-MDA5 antibody is mainly present in DM patients and is a strong predictor for poor prognosis diagnosis of DM with A/SIP and is an independent risk factor for death of ILD in DM.