目的：分析原发性胆汁性肝硬化(PBC)患者的临床特征,以提高对该病诊治的认识。方法：分析17例PBC患者的一般资料、临床表现、生化指标、免疫学指标及病理学改变。结果：PBC主要累及中年女性，易合并干燥综合征（SS）。胆管酶升高及抗线粒体M2抗体阳性有助于本病诊断。治疗首选熊去氧胆酸，糖皮质激素、免疫抑制剂适用于合并SS者。结论：早期诊断、早期治疗PBC是防止发展为终末期肝硬化的关键。
目的 探讨不同诊断或分类标准在脊柱关节病患者之间诊断效能、临床特点。 方法 收集2000年2月－2012年8月141例脊柱关节病患者及197例类风湿关节炎、未分化关节炎患者资料。采用3种诊断标准下对141例患者分为纽约标准（NY）组、欧洲脊柱关节病研究（ESSG）组及脊柱关节病国际评估（ASAS）组，统计患者的临床表现、骶髂关节CT、MRI、实验室指标，并加入197例类风湿关节炎未分化关节炎患者，计算和比较3种诊断的敏感性、特异性。 结果 在141例脊柱关节病中，62例符合NY标准，34例符合ESSG标准，45例只符合ASAS标准。3组患者在性别、胸廓受限、附着点炎及跖（指）炎、非甾体抗炎药及慢作用药使用、炎症因子水平方面差异无统计学意义（P＞0.05）；ASAS组则较19 NY组及ESSG组患者更年轻、病程较短，且表现有外周关节炎明显高于其余两组（P＜0.05）；NY组炎性腰背痛、脊柱活动受限、HLA-B27阳性率明显高于其余两组，且使用生物制剂比例最高，达48.38；在骨盆X线、骶髂关节CT表现关节侵蚀上，NY组较其他两组更严重。NY、ESSG、ASAS分类标准敏感性依次为43.97%、47.51%、73.76%，特异性依次为100.00%、90.86%、84.26%。 结论 ASAS分类标准敏感性高，患者更年轻、病程更短，炎性腰背痛、外周关节炎是ASAS诊断中非常重要的指标；NY组脊柱活动受限及关节侵蚀最显著，HLA-B27阳性可能是预测AS很好的指标。NY标准特异性最高，敏感性最低，而ASAS标准敏感性最高，特异性最低。
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.
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.
【摘要】 目的 提高对系统性红斑狼疮(SLE)并发急腹症(AA)临床复杂性的认识,总结诊治经验。方法 对2008年以来收治20例系统性红斑狼疮并发急腹症的患者进行回顾性分析,并复习近9年相关文献。结果 系统性红斑狼疮住院患者中并发急腹症发生率2.56％；急腹症多数(80%)与系统性红斑狼疮病情活动相关,也可能由独立于系统性红斑狼疮的其他疾病引起(20%),病情复杂,容易误诊。腹部CT尤其是增强CT检查对确定系统性红斑狼疮相关急腹症病因有重要作用。结论 系统性红斑狼疮活动是系统性红斑狼疮并发急腹症最主要的原因,SLEDAI评分在系统性红斑狼疮并发急腹症鉴别诊断中有一定作用。及时诊断、正确治疗后,系统性红斑狼疮活动相关急腹症患者的预后较好。
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.
【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.