ObjectiveTo analyze the incidence of heart disease, its clinical manifestations and risk factors in patients with polymyositis (PM) and dermatomyositis (DM). MethodWe collected the clinical data of 138 PM (n=78) and DM (n=60) patients treated between January 2008 and March 2014, among whom there were 64 males and 74 females with an average age of (48.5±19.6) years old, and an average disease course of (84.5±6.9) months. We analyzed their incidence of heart disease, its clinical manifestations and the risk factors. Subsequently, we adopted single-factor analysis to analyze such factors as age, gender, disease course, kinds of disease, creatine kinase (CK), CK-MB, CK-MB/CK, troponin T, antinuclear antibodies, anti-SSA antibody, erythrocyte sedimentation rate, C-reactive protein, disease activity score, muscle force, pulmonary interstitial lesions and pulmonary artery hypertension. ResultsThere were 59 (42.7%) patients with heart disease, 48 (34.8%) with abnormal electrocardiogram, and 52 (37.7%) with abnormal color Doppler ultrasound results. Logistics multiple factors regression analysis found that the course of the disease (OR=1.669, P=0.010), myositis disease activity score (OR=7.456, P<0.001), pulmonary interstitial lesions (OR=4.568, P=0.014) were risk factor for heart disease in PM/DM patients. ConclusionsLong disease course duration, high myositis activity score and pulmonary interstitial lesions are strong predictors for heart damage in PM/DM patients.
ObjectiveTo investigate the clinical characteristics of polymyositis (PM)/dermatomyositis (DM) with acute interstitial pneumonia (AIP) as the presenting symptoms, and identify characteristics of such disease. MethodsA retrospective analysis was conducted on the hospitalized patients with PM/DM with AIP as the presenting symptoms, from October 2009 to June 2015 in the Departemnt of Respiratory Medicine, Guangzhou Institute of Respiratory Diseases. ResultsThey were two males and six females with a mean age of 54.8±7.5 years. The common clinical features included fever (8 cases), shortness of breath (8 cases), rapidly progressive exertional dyspnea (8 cases), dry cough (6 cases), decreased muscle strength (8 cases), and typical rash (7 cases). Electromyography showed neurogenic or myogenic leision in these 8 cases. Muscle biopsy revealed myositis in 7 cases. High resolution CT (HRCT) revealed widespread ground glass patterns in all patients. All patients received noninvasive positive pressure mechanical ventilation on the first hospital day. High dose of methylprednisolone or combination with intravenous cyclophosphamide were initiated on 2.3±1.4 hospital day. Six patients survived to hospital discharge and two patient died. ConclusionsThe most common symptoms in patients of PM/DM with AIP are shortness of breath, progressive exertional dyspnea, and dry cough. Typical rash is seen in most of the patients.The diagnosis can be established by combinating the characteristics of HRCT, electromyography and muscle biopsy. Earlier intervention with noninvasive positive pressure mechanical ventilation and immunosuppressive may improve clinical outcome in patients of PM/DM with AIP.
Objective To evaluate the efficacy and safety of glucocorticoids (GC) monotherapy and GC combined with tacrolimus (TAC) therapy in patients with anti-synthetase syndrome-associated interstitial lung disease (ASS-ILD). Methods Through retrospective analysis and propensity score matching (PSM) analysis, the 2-year progression-free survival (PFS) and related side effects of ASS-ILD patients in TAC+GC group and GC monotherapy group were compared. Predictors associated with PFS were analyzed with COX. Results The 2-year PFS rate of TAC+GC group was better than that of GC group [P=0.0163; hazard ratio (HR) 0.347]; Univariate and multivariate analysis of the COX regression model for 2-year PFS in the two groups suggested that creatine kinase level (P=0.0019, HR 1.002) and initial treatment selection [(TAC+GC) vs. GC, P=0.0197, HR 0.207] were independent predictors of PFS; PSM analysis showed that the 2-year PFS rate of TAC+GC group (54.5%) was higher than that of GC group (18.2%) (P=0.0157, HR 0.275). In terms of adverse effect, there was no significant increase in GC+TAC group compared with GC group. Conclusion Compared with GC monotherapy, initial TAC+GC treatment significantly prolonged PFS in ASS-ILD patients and did not increase the incidence of drug-related complications.
ObjectiveTo study the survival rate and death cause of patients with polymyositis (PM) and dermatomyositis (DM). MethodsBased on the Bohan and Peter diagnosis standard, DM (n=52) and PM (n=98) hospitalized patients between January 1, 2008 and January 1, 2013 were chosen to be followed up to January 2013, or to their death. Sex, age, disease entities, course of the disease, muscle creatine enzyme, interstitial lung disease, connective tissue diseases, lung infection, cardiac involvement, respiratory muscle paralysis, JO-1 antibody, hypoalbuminemia, tumor, and long-term hormone and immune inhibitor treatment were the influencing factors of death. ResultsThirty-eight patients died during the follow-up period, and the 1-, 3- and 5-year survival rate were 87.7%, 74.5% and 55.9% respectively. Cox regression analysis showed that interstitial pneumonia (RR=12.119, P=0.001), heart disease (RR=2.935, P=0.020) and tumor (RR=3.735, P=0.048) were the unfavorable factors of death, while long-term hormones (RR=0.329, P=0.024) and persistent immunosuppressant therapy (RR=0.148, P=0.022) were protective factors. ConclusionThe five-year survival rate of patients with PM/DM is still low, and pulmonary interstitial disease, tumor, cardiac involvement, and pulmonary infection are the major dead causes, while long-team immunosuppression and hormone therapy can decrease the PM/DM mortality.
目的 研究活动期多发性肌炎患者外周血白细胞细胞因子信号转导蛋白抑制因子（SOCS）1、SOCS2、SOCS3和细胞因子诱导的含SH2区域蛋白1（CIS）与正常人表达的差异，探讨SOCS在多发性肌炎发病中可能的作用。 方法 2011年6月－12月，采用实时荧光定量聚合酶链反应法检测了14例活动期多发性肌炎患者和14例正常人外周血白细胞中SOCS1、SOCS2、SOCS3和CIS1基因的相对表达量。 结果 与对照组相比，多发性肌炎症患者外周血白细胞基因SOCS 1～3表达明显降低（P值均＜0.05），CIS1基因的表达较对照组明显升高（P＜0.05），差异有统计学意义。 结论 SOCS基因家族可能参与了多发性肌炎的发病，该蛋白分子家族的成员可能会成为多发性肌炎治疗的一种新的候选基因。
目的 研究活动期多发性肌炎患者外周血白细胞细胞因子信号转导蛋白抑制因子（SOCS）1、SOCS2、SOCS3和细胞因子诱导的含SH2区域蛋白1（CIS）与正常人表达的差异，探讨SOCS在多发性肌炎发病中可能的作用。 方法 2011年6月－12月，采用实时荧光定量聚合酶链反应法检测了14例活动期多发性肌炎患者和14例正常人外周血白细胞中SOCS1、SOCS2、SOCS3和CIS1基因的相对表达量。 结果 与对照组相比，多发性肌炎症患者外周血白细胞基因SOCS 1～3表达明显降低（P值均＜0.05），CIS1基因的表达较对照组明显升高（P＜0.05），差异有统计学意义。 结论 SOCS基因家族可能参与了多发性肌炎的发病，该蛋白分子家族的成员可能会成为多发性肌炎治疗的一种新的候选基因。
Thymoma complicated with polymyositis and myasthenia gravis is a rare case, which can be clearly diagnosed and given symptomatic treatment according to its own diagnostic criteria, imaging and laboratory examinations. This paper reports the clinical data of a thymoma patient with polymyositis and myasthenia gravis admitted to the Seventh Affiliated Hospital of Sun Yat-Sen University, and discusses the possible pathogenesis and treatment methods.
Objective To analyze the clinical characteristics of dermatomyositis ( DM) and polymyositis ( PM) with pulmonary involvement. Methods A retrospective study was performed in 27 DM/PM patients with pulmonary involvement, who were admitted to the First People’s Hospital of Kunming fromJanuary2001 to December 2009. The clinical manifestation, laboratory examination, chest high resolution CT ( HRCT) , pulmonary function test, treatment efficacy and prognosis were analyzed. Results In 27 DM/PM patients with pulmonary involvement, pulmonary manifestations occurred in 23 cases, such as cough ( 44% ) , expectoration ( 30% ) , and dyspnea ( 11% ) . Erythrocyte sedimentation rate, creatine kinase, C-reactive protein, and lactic dehydrogenase were significantly increased in 63% , 67% , 56% , and 44% of patients. Anti-Jo-1 antibody was positive in eight cases ( 29% ) . The electromyogram ( EMG) revealed myogenic changes in all patients. Pulmonary interstitial changes were the predominant HRCT manifestations. Pulmonary function test revealed mainly restrictive ventilation dysfunction and decreased diffusion capacity. Most patients had a good prognosis by glucocorticoid treatment. Conclusions For patients with DM/PM, especially who present nonspecific pulmonary symptoms, chest HRCT and pulmonary function test should be recommended as early screening tools.