Objective To analyze the clinical characteristics of antimitochondrial antibody (AMA)-associated cardiac injury. Methods AMA positive patients admitted to West China Hospital of Sichuan University between June 2008 and November 2023 were retrospectively selected. They were categorized into the simple cardiac involvement group and the cardiac and skeletal muscle involvement group according to the presence of skeletal muscle injury. Differences in demographic characteristics, serologic indices, cardiac structure and function, and arrhythmias were compared between the two groups. Results A total of 55 AMA-M2 positive patients with myocardial injury were enrolled. There were 18 cases in the simple cardiac involvement group and 37 cases in the cardiac and skeletal muscle involvement group among them. The age (P=0.002) and mortality rate (P=0.031) of the simple cardiac involvement group were higher than those of the cardiac and skeletal muscle involvement group. There were significant differences in biochemical indicators such as lactate dehydrogenase and α-hydroxybutyrate dehydrogenase between the two groups, and the levels of myocardial enzymes such as creatine kinase isoenzyme, myoglobin and troponin T in the cardiac involvement group were lower than those in the cardiac with skeletal muscle involvement group (P<0.05). Meanwhile, there were significant differences in the incidence of atrial premature beats, atrial fibrillation and other arrhythmias between the two groups (P<0.05). In terms of treatment modalities, glucocorticoids and immunosuppressants were used more frequently in the cardiac combined skeletal muscle involvement group than in the cardiac involvement alone group, whereas β-blockers and diuretics were more prevalent in the cardiac involvement alone group (P<0.05). Conclusion Patients with cardiac involvement alone may have a more insidious or rapid progression of the disease, which requires clinicians to pay higher attention to and provide timely and effective treatment, thus improving the overall prognosis and quality of life of the patients.
Objective To explore the clinical characteristics of patients with connective tissue disease with positive anti-small ubiquitin-like modifier activating enzyme (SAE) antibodies. MethodsRetrospectively select the patients who completed the screening of myositis autoantibodies in West China Hospital of Sichuan University between January 1, 2015 and May 30, 2021. Meanwhile, patients with positive anti-SAE antibodies were screened out. According to the clinical data of anti-SAE antibodies positive patients, they were divided into the following groups: tumor group and non-tumor group, ILD group and non-ILD group, inflammatory myopathy group and non-inflammatory myopathy group. Clinical symptoms, signs, laboratory examinations, imaging examinations and other clinical data of the above patients were collected. Results A total of 1 594 patients were screened for myositis autoantibodies, of which 56 were positive for anti-SAE antibodies, with a positive rate of 3.5%. In 56 patients, 32.1% in skin involvement, 35.7% in muscle involvement, 12.5% in joint involvement, 5.4% in dysphagia, 5.4% in weight loss, 58.9% in patients with interstitial lung disease (ILD) and 12.5% in patients with tumor history. There was no significant difference in age, sex, skin involvement, muscle involvement, joint involvement and respiratory system involvement between the tumor group and the non-tumor group (P>0.05). Except for age, the frequency of muscle involvement, and positive rate of anti-Ro-52 antibody, there was no significant difference in other indicators between the ILD group and the non-ILD group (P>0.05). Except for the positive rate of ILD, the frequency of skin involvement, the frequency of muscle involvement, the level of creatine kinase and hydroxybutyrate dehydrogenase (P<0.05), there was no significant difference in other indexes between the non-inflammatory myopathy group and the inflammatory myopathy group (P>0.05). Conclusions The patients with positive anti-SAE antibodies mainly present skin and muscle symptoms, and are prone to ILD, malignant tumor and dysphagia. Patients with positive anti-SAE antibodies and ILD were older, had less muscle damage, and had a higher positive rate of anti-Ro-52 antibody. Anti-SAE antibodies appear not only in patients with inflammatory myopathy, but also in non-inflammatory myopathy, often associated with a higher frequency of ILD and less muscle involvement.
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.
Idiopathic inflammatory myopathies are a group of connective tissue diseases characterized by nonsuppurative inflammation of the striated muscle. At present, the diagnostic criteria for polymyositis/dermatomyositis classification proposed by Bohan and Peter in 1976 is mainly used clinically. In clinical observations, it is found that myopathy involves not only skin and muscle but also affects many systems of the body. Interstitial lung disease occupies an important part, and it is an important cause of death of patients with inflammatory myopathy. Patients with idiopathic myositis should be examined as early as possible by high-resolution CT to improve the detection rate of myositis-associated interstitial lung disease and start treatment as soon as possible. At the same time, the patients with myositis have different clinical manifestations due to specific antibodies in the serum; some specific antibodies may indicate poor prognosis and poor treatment response. Timely screening of patients with positive myositis-specific antibodies in patients with the pulmonary interstitial disease can help the patient’s diagnosis and treatment process.
Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness with high serum muscle enzyme levels, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. The mechanism of necrotizing myopathy remains unclear. The new European Neuromuscular Centre criteria divides IMNM into three distinct subtypes according to different autoantibodies, which reminds us antibodies may be involved in the pathogenesis of IMNM and different subtypes may have different pathogenesis. This review summarizes the current understanding of the pathogenesis of IMNM.
Objective To present the pooled quantitative evidence of clinical features and current treatments of programmed death 1 (PD-1) / programmed death-ligand 1 (PD-L1) inhibitor-associated vasculitis. Methods Medline, Embase, EBM, CNKI, WanFang Data and VIP databases were searched for all available studies reporting PD-1/PD-L1 inhibitor-associated vasculitis till March 23, 2022. We summarized and systematically reviewed the included articles, and analyzed the data results with descriptive statistical methods. Results A total of 38 articles were included, including 43 patients. The median age [median (minimum, maximum)] was 62 (31, 89) years, and most of patients were male (64.3%). Lung cancer was the most common tumor (47.6%). The median onset time of vasculitis [median (minimum, maximum)] was 12 (1, 120) weeks after medication. Small vasculitis (62.8%) and cutaneous vasculitis (26.7%) were the most common types. The Common Terminology Criteria for Adverse Events of vasculitis was predominantly 3-4 (83.7%). After diagnosed with vasculitis, PD-1/PD-L1 inhibitors were discontinued in 81.6% of patients, and glucocorticoid was administrated in 88.4% of patients. After treatment, 90.0% of patients had significant improvement during follow-up. However, when the discontinuation of PD-1/PD-L1 inhibitors, 55.6% of patients tumor progressions, and 35.0% of patients dead. Conclusions Special attention should be paid to the occurrence of vasculitis when using PD-1/PD-L1 inhibitors for malignant tumor therapies. Stopping PD-1/PD-L1 inhibitors and using glucocorticoid are the essential methods to treat vasculitis, but the above treatments may bring a high risk of tumor progression.
Traditional antiphospholipid antibody detection exhibits significant limitations in the precise diagnosis and treatment of obstetric antiphospholipid syndrome (OAPS). This article systematically reviews the research progress of novel biomarkers in the field of OAPS, explores their clinical application prospects, and primarily summarizes the potential value of integrating the aforementioned indicators into a composite risk assessment model to better guide early risk stratification and individualized targeted treatment for high-risk pregnant women. It provides theoretical references for advancing OAPS towards precise management.
