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自身免疫性风湿疾病相关间质性肺疾病:来自土耳其的经验

Interstitial Lung Disease Associated with Autoimmune Rheumatic Diseases: An Experience from Türkiye.

作者信息

Bes Cemal, Yildirim Fatih, Eren Ramazan, Ulusan Şerife, Deniz Rabia, Karaalioglu Bilgin, Ozgur Duygu Sevinç, Akkuzu Gamze, Demirkol Barış, Erener Mustafa Kağan, Yalçın Mutlu Melek, Çetinkaya Erdoğan

机构信息

Department of Rheumatology, University of Health Sciences, Başakşehir Çam and Sakura City Hospital, İstanbul, Türkiye.

Department of Chest Disease, University of Health Sciences, Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, İstanbul, Türkiye.

出版信息

Eur J Rheumatol. 2025 Jul 31;12(3):1-6. doi: 10.5152/eurjrheum.2025.24117.

Abstract

Objective: Interstitial lung disease (ILD) is one of the most challenging involvement of autoimmune rheumatic diseases (ARDs) and could lead to significant morbidity and mortality. In this article, a collaborative work of tertiary rheumatology and pulmonology centers describing demographic, serological, and radiological findings of patients with ARD associated with ILD (ARD-ILD) is presented. Methods: A descriptive, retrospective study, and data related to demographics, clinical, laboratory, radiologic, or histopathological findings of ILD were collected from the study participants' charts. Results: Around 212 patients with ARD-ILD were evaluated. Of the patients, 172 (81.1%) were female and 40 (18.9%) were male. The distribution of the rheumatic diseases was as follows: systemic sclerosis in 114 (53.8%), rheumatoid arthritis in 47 (22.2%), Sjögren's syndrome in 14 (6.6%), inflammatory myopathy in 16 (7.5%) patients, interstitial pneumonia with autoimmune features (IPAF) in 9 (4%) patients, undifferentiated connective tissue disease in 8 (3.8%), and systemic lupus erythematosus in 4 (1.9%). According to the radiological patterns, 71.7% of the patients had nonspecific interstitial pneumonia (NSIP), 13.7% had definite usual interstitial pneumonia (UIP), 8.5% had probable UIP, 3.8% had lymphocytic interstitial pneumonia, 1.9% had organizing pneumonia, and 0.5% had an atypical pattern. Conclusion: This study showed that the most common rheumatic disease causing ILD is still systemic sclerosis, and NSIP is more prominent as a radiological pattern. IPAF, a disease that has entered the literature in recent years, is also an important type of ILD. Given the multisystemic involvement of ARDs, collaboration among different disciplines is undoubtedly crucial in the diagnosis and management of these diseases.

摘要

目的

间质性肺疾病(ILD)是自身免疫性风湿性疾病(ARDs)中最具挑战性的累及部位之一,可导致显著的发病率和死亡率。本文展示了三级风湿病学和肺病学中心的一项合作研究,描述了与ILD相关的ARD(ARD-ILD)患者的人口统计学、血清学和放射学特征。方法:进行一项描述性、回顾性研究,从研究参与者的病历中收集与ILD的人口统计学、临床、实验室检查、放射学或组织病理学特征相关的数据。结果:共评估了约212例ARD-ILD患者。其中,172例(81.1%)为女性,40例(18.9%)为男性。风湿性疾病的分布如下:系统性硬化症114例(53.8%),类风湿关节炎47例(22.2%),干燥综合征14例(6.6%),炎性肌病16例(7.5%),具有自身免疫特征的间质性肺炎(IPAF)9例(4%),未分化结缔组织病8例(3.8%),系统性红斑狼疮4例(1.9%)。根据放射学表现,71.7%的患者为非特异性间质性肺炎(NSIP),13.7%为明确的普通型间质性肺炎(UIP),8.5%为可能的UIP,3.8%为淋巴细胞性间质性肺炎,1.9%为机化性肺炎,0.5%为非典型表现。结论:本研究表明,导致ILD最常见的风湿性疾病仍是系统性硬化症,NSIP作为放射学表现更为突出。IPAF是近年来进入文献的一种疾病,也是ILD的一种重要类型。鉴于ARDs的多系统累及,不同学科之间的合作在这些疾病的诊断和管理中无疑至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9b99/12362481/88edd97a3600/ejr-12-3-24117_f001.jpg

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