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 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.
【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.
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.
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 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 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 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.
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。