Melanoma differentiation associated gene 5 (MDA5) antibody is a kind of myositis-specific autoantibodies, which can be used as a biological marker for dermatomyositis (DM) patients. It has been shown that this antibody is closely related to the occurrence of interstilung disease (ILD) in DM patients, which may lead to rapidly progressive interstitial lung disease in DM patients with MDA5 antibody positive, leading to a higher mortality rate. Therefore, the early detection, diagnosis and treatment of this disease have attracted the attention of clinical workers. In this paper, the progress of diagnosis and treatment of MDA5 antibody-positive DM with ILD is reviewed.
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.
Objective To evaluate the clinical relationship between serum carcinoembryonic antigen (CEA) and mortality of anti-melanoma differentiation associated gene 5 (MDA5) antibody positive dermatomyositis with interstitial lung disease (ILD). MethodsThe consecutive clinical data of 214 patients with anti MDA5 antibody positive dermatomyositis from West China Hospital of Sichuan University from February 2017 to September 2019 were collected retrospectively, including demographic, laboratory examination and imaging examination data. Patients were divided into CEA elevated group (CEA≥4.63 ng/mL) and CEA normal group (CEA<4.63 ng/mL) according to CEA level. R4.1.2 software was used for statistical analysis of all data, and Kaplan Meier method was used to draw the survival curve. Cox proportional hazard model was used to analyze the survival of patients with ILD, and to explore the risk factors associated with the survival of patients with anti-MDA5 antibody positive dermatomyositis with ILD. Results There were 180 patients with ILD who met the inclusion and exclusion criteria, 57 patients with rapidly progressive pulmonary interstitial fibrosis (RPILD), and 123 patients without RPILD; 121 women and 59 men, with an average age of 50.2±10.7 years; The average follow-up was 23.5 months, and 52 patients died. Univariable analysis suggested that CEA≥4.63 ng/mL, smoking, RPILD, lactate dehydrogenase (LDH) ≥321 IU/L, albumin<30 g/L and dyspnea were risk factors associated with death in patients with anti MDA5 dermatomyositis combined with ILD. Multivariable Cox regression analysis showed that CEA≥4.63 ng/mL [hazard ratio (HR) =3.01, 95% confidence interval (CI) 1.23 - 7.32, P=0.015], RPILD (HR=3.87, 95%CI 2.09 - 7.19, P<0.001), smoking (HR=2.37, 95%CI 1.25 - 4.47, P=0.008), LDH≥321 IU/L (HR=2.47, 95%CI 1.23 - 4.96, P=0.011), albumin<30 g/L (HR=2.57, 95%CI 1.38 - 4.78, P=0.003) were independent predictors for mortality. ConclusionsSerum CEA level can be used as a clinical prognostic predictor in patients with anti-MDA5 positive dermatomyositis and ILD. RPILD, smoking, LDH≥321 IU/L, and albumin<30 g/L are independent predictors for mortality.
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.
ObjectiveTo analyze the clinical characteristics of patients with amyopathic dermatomyositis with organizing pneumonia (ADM-OP).MethodsThe clinical data of 8 patients hospitalized with ADM-OP from June 2014 to June 2018 were retrospectively reviewed and simultaneously compared with those of 8 patients of cryptogenic organizing pneumonia (COP).ResultsThe incidence of skin lesion, Gottron’s sign, mechanic’s hand and positive anti-synthase antibodies in the ADM-OP patients were 87.5%, 87.5% 75.0% and 87.5% respectively. Gender, smoking, respiratory symptoms and signs, arterial partial pressure of oxygen, arterial partial pressure of carbon dioxide and treatment strategy were no statistical difference between ADM-OP and COP patients, but the onset age and Chest CT fibrosis scores (CTFS) on admission existed differences. After treatment for 3 months, CTFS, rate of change and forced vital capacity (FVC) existed differences. After treatment for 6 months, CTFS, rate of change, FVC and diffusing capacity of the lung for carbon monoxide existed differences.ConclusionsSkin lesion, Gottron’s sign, mechanic’s hand and positive anti-synthase antibodies are more common in ADM-OP patients. Their response to treatment is good but the improvement rates in CTFS and pulmonary function are slower than those of COP patients.
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.
目的：探討無肌病性皮肌炎的臨床特點。方法：回顧性分析符合Euwer提出的ADM診斷標準的5例患者的臨床資料，包括臨床癥狀體征、肌酶譜、肌電圖、肌肉病理檢查、胸部影像學檢查、治療方案。結果：所有患者均先后出現皮肌炎典型皮損，無肌痛或肌無力等主訴，肌酶譜、肌電圖均正常。2例抗核抗體陽性，3例有肌活檢非特異性改變，1例合并間質性肺炎，無患者合并惡性腫瘤。結論：皮肌炎是一種復雜的綜合征，需要行全身系統檢查，結合肌電圖和肌肉病理檢查進行綜合診斷。
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.
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.
【摘要】 目的 探討新診斷的多發性肌炎（PM）、皮肌炎（DM）和無肌病性皮肌炎（ADM）肺部病變發生率、臨床特點及相關因素。 方法 回顧性分析2008年1月—2010年7月新確診的206例PM、DM、ADM臨床表現、肺部影像學、肺功能、超聲心動圖和實驗室指標。 結果 206例患者中合并肺部病變156例，以肺間質病變（ILD）最多見，占患者總數的51.46%。在性別、病程、是否吸煙方面合并ILD與無ILD患者相比差異無統計學意義，合并ILD患者年齡大于無ILD組。合并ILD患者呼吸困難、發熱、雷諾現象、關節炎或關節痛概率增加。合并ILD組白蛋白低于無ILD組，血沉和免疫球蛋白IgM高于無ILD組。急性ILD組中女性患者及出現雷諾現象的概率高于慢性組。206例患者中死亡13例，其中周圍型肺癌1例，特發性血小板減少并顱內出血1例，嚴重肺部感染11例；死亡患者中10例伴肺間質纖維化。合并急性ILD患者死亡率較慢性組高2倍。 結論 PM、DM、ADM患者肺部病變發生率高，以ILD多見，發熱、年齡大、白蛋白降低、血沉升高、雷諾現象及關節炎或關節痛都是合并ILD的相關因素。合并急性ILD患者預后差，死亡者常合并肺部感染。【Abstract】 Objective To explore the prevalence, clinical features, and predictive factors of pulmonary involvement in newly diagnosed polymyositis (PM), dermatomyositis (DM) and amyopathic dermatomyositis (ADM), in order to carry out early diagnosis and treatment, and improve the prognosis. Methods The clinical manifestations, chest imaging, pulmonary function test, ultrasonic cardiography and laboratory results of 206 inpatients with PM, DM and ADM in West China Hospital of Sichuan University from January 2008 to July 2010 were reviewed retrospectively. Results One hundred and fifty-six out of 206 patients developed PM/DM/ADM associated lung diseases, including 106 cases (51.46%) of interstitial lung disease (ILD). There was no significant difference in gender, disease duration, and smoking or not between the ILD and non-ILD group, but patients in the ILD group were significantly older than non-ILD group. The results also showed that patients with ILD were much more likely to have symptoms of breathing difficulties, fever, Raynaud phenomenon and arthritis/arthralgia. The patients with ILD had lower level of albumin but higher levels of ESR and IgM; In the group of acute ILD, female patients and the ratio of Raynaud phenomenon were higher than those in the chronicity group. Of the 206 patients, 13 patients died, including 1 death of peripheral lung cancer, 1 of essential thrombocytopenia and intracranial hemorrhages, and 11 of severe lung infection, and 10 in these patients developed ILD. Mortality in patients with acute ILD was 2 times higher than the chronicity group. Conclusion The prevalence of lung diseases is high in patients with PM, DM and ADM. ILD is the main pulmonary involvement, and fever, older age at onset, hypoalbuminemia, higher values of blood sedimentation, Raynaud phenomenon and arthritis or arthralgia were the predictive factors for developing ILD. Patients with acute ILD have poor prognosis. Death cases often have pulmonary infections.