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 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.
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.
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.