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.
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 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 探讨皮肌炎合并乳腺恶性肿瘤的临床特征、诊治及预后情况。方法 回顾性分析我科自2008年以来收治的3例皮肌炎合并乳腺恶性肿瘤患者的临床资料,就两者关系结合文献进行分析。结果 3例皮肌炎合并乳腺恶性肿瘤患者中2例行乳腺癌改良根治术,病理报告为浸润性导管癌(淋巴结14/21枚转移)和原位癌(淋巴结0/17枚转移,伴4枚癌结节),因术后不能耐受静脉化疗,予内分泌治疗。1例行乳房肿瘤切除术及腋窝淋巴结活检术,2个月后因肺间质病死亡。结论 女性皮肌炎患者易伴发乳腺恶性肿瘤且预后差,对明确诊断为皮肌炎的女性患者有必要进行乳腺恶性肿瘤的筛选。
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.
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.
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.
【摘要】 目的 探讨新诊断的多发性肌炎（PM）、皮肌炎（DM）和无肌病性皮肌炎（ADM）肺部病变发生率、临床特点及相关因素。 方法 回顾性分析2008年1月—2010年7月新确诊的206例PM、DM、ADM临床表现、肺部影像学、肺功能、超声心动图和实验室指标。 结果 206例患者中合并肺部病变156例，以肺间质病变（ILD）最多见，占患者总数的51.46%。在性别、病程、是否吸烟方面合并ILD与无ILD患者相比差异无统计学意义，合并ILD患者年龄大于无ILD组。合并ILD患者呼吸困难、发热、雷诺现象、关节炎或关节痛概率增加。合并ILD组白蛋白低于无ILD组，血沉和免疫球蛋白IgM高于无ILD组。急性ILD组中女性患者及出现雷诺现象的概率高于慢性组。206例患者中死亡13例，其中周围型肺癌1例，特发性血小板减少并颅内出血1例，严重肺部感染11例；死亡患者中10例伴肺间质纤维化。合并急性ILD患者死亡率较慢性组高2倍。 结论 PM、DM、ADM患者肺部病变发生率高，以ILD多见，发热、年龄大、白蛋白降低、血沉升高、雷诺现象及关节炎或关节痛都是合并ILD的相关因素。合并急性ILD患者预后差，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.
ObjectiveTo investigate the prognostic relevance of serum triglyceride (TG) levels in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis-associated interstitial lung disease (ILD). Methods A retrospective data collection was conducted on patients diagnosed with anti-MDA5 antibody-positive dermatomyositis-associated ILD at West China Hospital of Sichuan University between February 2017 and July 2021. The clinical data, laboratory tests, and imaging examinations were collected, and the patients were followed up. According to the survival and death status of patients, they were divided into survival group and death group. According to TG levels, the patients were divided into a TG high level group and a TG low level group. We employed Cox proportional hazard models to investigate the variables linked to the mortality of individuals afflicted with anti-MDA5 antibody-positive dermatomyositis-associated ILD. Results A total of 204 patients with anti-MDA5 antibody-positive dermatomyositis-associated ILD were included. Among them, whose age ranged from 30 to 81 years old, with an average of (49.5±11.8) years old, there were 69 males and 135 females, 53 deaths and 151 survivors, 57 cases of rapidly progressive pulmonary interstitial fibrosis (RPILD) and 47 cases of non-RPILD. The results of multivariate Cox regression analysis showed that TG≥1.65 mmol/L, combined with RPILD, combined with dyspnea, age, lactate dehydrogenase≥321 U/L, and albumin<30 g/L were independent factors affecting the long-term prognosis of patients (P<0.05). The Kaplan-Meier method analysis results showed that the survival rate of the TG high level group was lower than that of the TG low level group (P=0.032). Conclusions Elevated TG levels can serve as a clinical indicator of adverse prognosis in patients with dermatomyositis-associated ILD who exhibit positive anti-MDA5 antibody status. Additionally, age, comorbidity with RPILD, combined with dyspnea, lactate dehydrogenase≥321 U/L, and albumin<30 g/L are independent factors contributing to the increased mortality risk among individuals with dermatomyositis-associated ILD who test positive for anti-MDA5 antibody.