1997-2024年西班牙特发性炎症性肌病的流行病学、自身免疫重叠及院内结局

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中文摘要

目的： 明确西班牙特发性炎症性肌病（IIM）的流行病学、自身免疫重叠及院内结局，将真正的IIM与症状性肌病编码（G73.7）区分开，并识别死亡和重症监护（ICU）入院的预测因素。
方法： 基于西班牙出院登记数据库（CMBD和RAE-CMBD，1997-2024年）的人群分析。率为住院率，而非发病率或患病率。真正的IIM包括DM/PM组（皮肌炎、多发性肌炎和副肿瘤性DM编码）、免疫介导性坏死性肌病/其他（IMNM/其他）以及包涵体肌炎（IBM）。估计值经患者聚类标准误验证。
结果： 在36 732例次（2016-2024年8053例患者，更早时期9162例）中，34 228例次（93.2%）为真正的IIM（DM/PM组84.0%：61.4%编码为DM，35.7%编码为PM，2.9%为副肿瘤性；IMNM/其他13.7%，IBM 2.4%）。住院率从每10万人年1.67升至4.24，仅限于≥60岁人群。13.7%（约七分之一）合并自身免疫性疾病。死亡率为6.1%且保持稳定。死亡的预测因素为高龄（校正OR最高达7.97）、恶性肿瘤、心力衰竭、间质性肺病（ILD）、吞咽困难和慢性肾脏病（C统计量0.77）；ICU入住率在高龄老人中下降（OR 0.21），提示治疗上限降级。COPD和糖尿病的表观保护效应反映了碰撞偏倚。在DM/PM组内，29.4%被编码为多发性肌炎（M33.2），其ILD、重叠和抗合成酶特征更多但恶性肿瘤更少，该比例从40%降至27%。
结论： 28年间IIM住院率翻倍，驱动因素为IMNM的识别和人口老龄化，结局由急性并发症和合并症而非亚型决定。症状性肌病应单独分析，副肿瘤性皮肌炎应与特发性DM统一。

英文摘要

OBJECTIVES: To define the epidemiology, autoimmune overlap and in-hospital outcomes of idiopathic inflammatory myopathies (IIM) in Spain, separating true IIM from the symptomatic-myopathy code (G73.7), and to identify predictors of mortality and intensive-care (ICU) admission.
METHODS: Population-based analysis of the Spanish hospital-discharge registries (CMBD and RAE-CMBD, 1997-2024). Rates are hospitalization rates, not incidence or prevalence. True IIM comprised the DM/PM group (dermatomyositis, polymyositis and paraneoplastic DM codes), immune-mediated necrotizing myopathy/other (IMNM/Other) and inclusion body myositis (IBM). Estimates were verified against patient-clustered standard errors.
RESULTS: Of 36 732 episodes (8053 patients in 2016-2024, 9162 earlier), 34 228 (93.2%) were true IIM (DM/PM group 84.0%: 61.4% DM-coded, 35.7% PM-coded, 2.9% paraneoplastic; IMNM/Other 13.7%, IBM 2.4%). Hospitalization rose from 1.67 to 4.24 per 100 000/year, confined to ages ≥60. Coexisting autoimmune disease occurred in 13.7% (about one in seven). Mortality was 6.1% and stable. Predictors of death were advancing age (adjusted OR up to 7.97), malignancy, heart failure, interstitial lung disease (ILD), dysphagia and chronic kidney disease (C-statistic 0.77); ICU admission fell in the very elderly (OR 0.21), indicating ceiling-of-care de-escalation. Apparent protective effects of COPD and diabetes reflected collider bias. Within the DM/PM group, 29.4% were coded as polymyositis (M33.2), with more ILD, overlap and antisynthetase features but less malignancy, declining from 40% to 27%.
CONCLUSION: IIM hospitalizations doubled over 28 years, driven by IMNM recognition and ageing, with outcomes governed by acute complications and comorbidity rather than subtype. Symptomatic myopathy should be analysed separately and paraneoplastic dermatomyositis unified with idiopathic DM.