目的:探讨合并血栓栓塞的肌炎患者的临床及免疫学特征。方法:回顾性分析2003—2019年于北京大学人民医院住院的390例肌炎患者的病历资料,包括人口学特征、皮肤、肌肉表现、脏器受累、实验室指标(肌酶、白蛋白、凝血功能、炎性指标、肌炎特异性/相关性抗体)、治疗方案,并根据有无合并血栓栓塞将患者进行分组比较,应用Logistic回归分析明确肌炎合并血栓栓塞的危险因素。结果:肌炎患者平均起病年龄(49.6±13.4)岁,男女比例为0.31:1,血栓栓塞发生率为4.62%(18/390),其中,55.6%(10/18)为下肢深静脉血栓形成,其次为脑梗塞(22.2%,4/18)、肺栓塞(11.1%,2/18)、肾动脉栓塞(5.6%,1/18)、上肢静脉血栓形成(5.6 %,1/18)。血栓栓塞发生时间方面,38.9%(7/18)发生在肌炎诊断的前后6个月内,50%(9/18)发生在肌炎确诊后。合并血栓栓塞的肌炎患者起病年龄偏大,平均(58.3±11.7)岁,合并糖尿病(44.4% vs. 16.4%,P=0.006)、冠心病(22.2% vs. 3.0%,P=0.003)、 90 d内手术史(16.7% vs. 3.5%,P=0.032)等并发症均高于无血栓栓塞组。实验室检查方面,合并血栓栓塞的肌炎患者C-反应蛋白(12.2 mg/L vs. 4.1 mg/L,P<0.001)、血清铁蛋白(20 085.5 μg/L vs. 216.6 μg/L,P<0.001)、血清白蛋白水平(32.4 g/L vs. 36.5 g/L,P=0.002)、D-二聚体(529.0 μg/L vs. 268.0 μg/L,P=0.002)均明显升高,活化部分凝血酶原时间(26.9 s vs. 28.7 s,P=0.049)显著低于无血栓栓塞组。肌炎发生血栓栓塞的危险因素包括:白蛋白水平降低(OR=0.831,95%CI:0.736~0.939,P=0.003)、合并糖尿病(OR=4.468,95%CI:1.382~14.448,P=0.012)、合并冠心病(OR=22.079,95%CI:3.589~135.837,P=0.001)。未见肌炎特异性抗体与血栓栓塞发生的相关性。结论:肌炎患者可发生血栓栓塞事件,合并白蛋白水平降低、糖尿病、冠心病的肌炎患者发生血栓栓塞的风险高,临床应高度警惕。

Objective: To investigate and analyse the clinical and immunological features of patients with myositis complicated with thromboembolism. Methods: We identified a cohort of 390 myositis patients diagnosed with myositis admitted to People’s Hospital of Peking University from 2003 to 2019. The patients were retrospectively enrolled in this investigation. According to the outcome of the color Doppler ultrasound, CT pulmonary angiography, pulmonary ventilation and perfusion scan patients were divided into myositis with and without thromboembolism group. Demographic, clinical (heliotrope rash, Gottron’s sign/papules, periungual erythema, skin ulceration, subcutaneous calcinosis, Mechanic’s hands, myalgia, interstitial lung disease, pulmonary arterial hypertension), laboratory, immunological [anti-autoantibodies including melanoma differentiation associated gene 5 (anti-MDA5), anti-Mi-2, anti-transcription intermediary factor-1γ (anti-TIF-1γ, anti-nuclear matrix protein 2 (anti-NXP2), anti-small ubiquitin-like modifier activating enzyme (anti-SAE), anti-synthetase], imaging and therapeutic status data of the patients at the diagnosis of myositis with and without thromboembolism were collected and the differences in these data were analyzed. Logistic regressive analysis was used to identify the risk factors of thromboembolism. Results: In the retrospective study, 390 myositis patients were investigated. The mean age of onset was (49.6±13.4) years, male to female ratio was 0.31:1. Thromboembolism was identified in 4.62% (18/390) of the myositis patients, which was lower than the published reports. Out of 18 patients with thromboembolism, 55.6% (10/18) of them were deep venous thrombosis, followed by cerebral infarction (22.2%, 4/18), pulmonary embolism (11.1%, 2/18), renal artery embolism (5.6%, 1/18) and embolism of upper extremity (5.6%, 1/18). Fifty percent of thromboembolism events occurred 6 months after the diagnosis of myositis, 38.9% of thromboembolism events occurred 6 months within the diagnosis of myositis, 11.1% of thromboembolism events occurred 6 months before the diagnosis of myositis. As compared with the myositis patients without thromboembolism, the myositis patients complicated with thromboembolism were older [(58.3±11.7) years vs. (49.3±13.4) years, P=0.006]. C-reaction protein (CRP) (12.2 mg/L vs. 4.1 mg/L, P<0.001), ferritin (20 085.5 μg/L vs. 216.6 μg/L, P<0.001) and D-dimer (529.0 μg/L vs. 268.0 μg/L, P=0.002) were significantly higher in thromboembolism group. Diabetes (44.4% vs. 16.4%, P=0.006), coronary heart disease (22.2% vs. 3.0%, P=0.003) and surgery (16.7% vs. 3.5%, P=0.032) were observed more common in thromboembolism group than those without thromboembolism. Activated partial thromboplastin time (APTT) (26.9 s vs. 28.7 s, P=0.049) and albumin (32.4 g/L vs. 36.5 g/L, P=0.002) was lower in thromboembolism group. The risk factors of thromboembolism in the myositis patients were low level of albumin (OR=0.831, 95%CI: 0.736-0.939, P=0.003), diabetes (OR=4.468, 95%CI: 1.382-14.448, P=0.012), and coronary heart disease (OR=22.079, 95%CI: 3.589-135.837, P=0.001) were independent significant risk factors for thromboembolism in the patients with myositis. There was no significant difference in clinical manifestations, myositis-specific antibodies or myositis-associated antibodies between the two groups. Conclusion: Thromboembolism is a complication of myositis. Lower levels of albumin, diabetes, and coronary heart disease might be risk factors of thromboembolism in myositis patients.