Clinical features and prognostic factors of systemic sclerosis in Guangxi, China: Retrospective, single-center study of long-term survival in 470 patients
Clinical features and prognostic factors of systemic sclerosis in Guangxi, China: Retrospective, single-center study of long-term survival in 470 patients
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中国广西系统性硬化症的临床特征和预后因素：470 例患者长期生存的回顾性、单中心研究
DOI：
10.1111/1756-185x.14261
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发表时间：
2021-12-10
影响因子：
2.5
通讯作者：
Lei, Ling
中科院分区：
文献类型：
--
作者：
Chen, Juan;Yang, Chenxi;Lei, Ling
目的：系统性硬化症（SSc）是一种自身免疫性疾病，其患病率因人群而异。我们对广西SSc患者进行分析，以提高临床对该病的认识。方法分析我院2021年10月至2019年1月收治的470例SSc患者的资料。采用Kaplan-Meier生存分析分析这些患者的特征。采用Cox比例风险回归分析确定预后因素。结果平均年龄50.44±12.31岁，女性285例（60.6%），尘肺2.1%，肺间质性疾病（ILD） 58.2%，肺动脉高压（PH） 18.7%，肾危重3.6%。这些患者有弥漫性皮肤系统性硬化症（dcSSc, 70.2%）或局限性皮肤系统性硬化症（29.7%），且PH和肾危象在dcSSc组更为常见。50岁及以上的患者ILD、PH和肌肉骨骼损伤的患病率更高，实验室生物标志物的阳性率更高，死亡率也更高（均P < 0.05）。74例（15.7%）死亡。非幸存者年龄较大，病程较长，ILD、限制性通气功能障碍、PH和肾危像的患病率较高，肌酸激酶心肌带（CK-MB）、c反应蛋白和免疫球蛋白A水平较高（均P < 0.05）。肾危象、PH和高CK-MB是死亡的独立危险因素。结论该地区SSc患者尘肺病发生率高于普通人群。我们的患者的10年累积生存率为74.9%，高于美国报道的患者。肾危象、PH和高CK-MB水平是死亡的独立危险因素。
Objective Systemic sclerosis (SSc) is an autoimmune disease the prevalence of which varies among populations. We analyzed SSc patients from Guangxi to improve the clinical understanding of this disease. Methods Data of 470 SSc patients admitted to our institution from October 2021 to January 2019 were examined. The characteristics of these patients were analyzed using Kaplan-Meier survival analysis. Cox proportional-hazard regression was used to identify prognostic factors. Results The average age was 50.44 +/- 12.31 years, 285 patients (60.6%) were women, 2.1% had pneumoconiosis, 58.2% had pulmonary interstitial disease (ILD), 18.7% had pulmonary hypertension (PH), and 3.6% had renal crisis. These patients had diffuse cutaneous systemic sclerosis (dcSSc, 70.2%) or limited cutaneous systemic sclerosis (29.7%), and PH and renal crisis were more common in the dcSSc group. Patients 50 years old or more had greater prevalences of ILD, PH, and musculoskeletal damage, greater positivity of laboratory biomarkers, and increased mortality (all P < .05). Seventy-four patients (15.7%) died. The non-survivors were older, had longer disease duration, had higher prevalences of ILD, restrictive ventilation dysfunction, PH, and renal crisis, and had higher levels of creatine kinase myocardial band (CK-MB), C-reactive protein, and immunoglobin A (all P < .05). Renal crisis, PH, and high CK-MB were independent risk factors for death. Conclusions Pneumoconiosis was more common in SSc patients than the general population from this region. Our patients had a 10-year cumulative survival rate of 74.9%, higher than reported for patients from the US. Renal crisis, PH, and high CK-MB level were independent risk factors for death.