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.
Objective To improve the vigilance and awareness of malignancy presenting as dermatosis and reduce misdiagnosis. Methods Two cases of gastric cancer presenting as dermatomyositis and erythroderma respectively in the last two years were retrospectively analyzed and the relevant literatures were reviewed. Results The two patients were admitted to hospital due to skin diseases， diagnosis of gastric cancer through endoscopy， and proved to be gastric cancer associated with dermatosis by pathological examination after surgical resection. Conclusions Paraneoplastic dermatoses can be seen as an early manifestation of the internal malignancy. The patients with paraneoplastic dermatoses should be excluded visceral tumors by the means of biomarkers， endoscopy， PET/CT， and so on.
目的：探讨无肌病性皮肌炎的临床特点。方法：回顾性分析符合Euwer提出的ADM诊断标准的5例患者的临床资料，包括临床症状体征、肌酶谱、肌电图、肌肉病理检查、胸部影像学检查、治疗方案。结果：所有患者均先后出现皮肌炎典型皮损，无肌痛或肌无力等主诉，肌酶谱、肌电图均正常。2例抗核抗体阳性，3例有肌活检非特异性改变，1例合并间质性肺炎，无患者合并恶性肿瘤。结论：皮肌炎是一种复杂的综合征，需要行全身系统检查，结合肌电图和肌肉病理检查进行综合诊断。
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.
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 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 describe the clinical characteristics of polymyositis/dermatomyositis (PM/DM) with anti-aminoacyl-tRNA synthetase (ARS) antibody positive. Methods The clinical, laboratory and radiographic results of PM/DM patients hospitalized in our department from September 2014 to November 2017 were retrospectively analyzed. Results A total of 39 patients were diagnosed (14 cases positive for anti-Jo-1 antibody, 10 cases positive for non-anti-Jo-1 ARS antibodies, and 15 negative for ARS antibodies). The frequency of ARS antibodies positive patients who had interstitial lung disease was higher than those patients without ARS antibodies (P<0.05). Amyosthenia and mechanic's hand were more common in the patients with anti-Jo-1 positive (P<0.05) and the frequency of clinical amyopathic dermatomyositis in non-anti-Jo-1 positive patients was significantly higher (P<0.05). Conclusions The clinical characteristics are similar between anti-Jo-1-positive and non-Jo-1 ARS antibodies positive patients. Most PM/DM patients carrying anti-Jo-1 antibodies with interstitial lung disease own typical imaging characteristics of nonspecific interstitial pneumonia overlap organizing pneumonia (NSIP/OP). It can be diagnosed of non-anti-Jo-1 antibody syndrome although there is no clinical manifestation of myositis and anti-jo-1 antibody is negative.
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 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.