具有自身免疫特征的“普通型”间质性肺炎的模式与进展：与具有自身免疫特征的经典间质性肺炎及特发性肺纤维化患者的比较。

The Pattern and Progression of "Usual" Interstitial Pneumonia with Autoimmune Features: Comparison with Patients with Classic Interstitial Pneumonia with Autoimmune Features and Idiopathic Pulmonary Fibrosis.

作者信息

Libra Alessandro, Colaci Michele, Spicuzza Lucia, Luca Giuliana, Fischetti Sefora, Pashalidis Giorgio, Ferrara Chiara Alfia, Ielo Giuseppe, Sambataro Domenico, La Rosa Giuliana, Libra Federica, Palmucci Stefano, Vancheri Carlo, Sambataro Gianluca

机构信息

Department of Clinical and Experimental Medicine, Regional Referral Center for Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 95123 Catania, Italy.

Internal Medicine Unit, Department of Clinical and Experimental Medicine, Division of Rheumatology, Cannizzaro Hospital, University of Catania, 95123 Catania, Italy.

出版信息

J Clin Med. 2024 Jan 10;13(2):369. doi: 10.3390/jcm13020369.

BACKGROUND

We proposed the term "UIPAF" to define patients with Usual Interstitial Pneumonia (UIP) associated with only one domain of the classification called "Interstitial Pneumonia with Autoimmune Features" (IPAF). The objective of this study was to evaluate the clinical presentation and prognosis of UIPAF patients, compared with two cohorts, composed of IPAF and idiopathic pulmonary fibrosis (IPF) patients, respectively.

METHODS

The patients were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and radiological data and evaluated by a multidisciplinary team.

RESULTS

We enrolled 110 patients with IPF, 69 UIPAF, and 123 IPAF subjects. UIPAF patients were similar to IPAF regarding autoimmune features, except for the prevalence of Rheumatoid Factor in UIPAF and anti-SSA in IPAF. A similar proportion of the two cohorts progressed toward a specific autoimmune disease (SAD), with differences in the kind of SAD developed. The real-life management and prognosis of UIPAF patients proved to be almost identical to IPF.

CONCLUSIONS

UIPAF shared with IPAF similar autoimmune features, suggesting the opportunity to be considered IPAF, excluding the morphological domain by the classification. However, the real-life management and prognosis of UIPAF are similar to IPF. These data suggest a possible modification in the therapeutic management of UIPAF.

背景

我们提出了“UIPAF”这一术语，用于定义仅与分类中的一个领域“具有自身免疫特征的间质性肺炎”（IPAF）相关的普通型间质性肺炎（UIP）患者。本研究的目的是评估UIPAF患者的临床表现和预后，并与分别由IPAF患者和特发性肺纤维化（IPF）患者组成的两个队列进行比较。

方法

根据临床、血清学和放射学数据将患者纳入IPAF、UIPAF或IPF组，并由多学科团队进行评估。

结果

我们纳入了110例IPF患者、69例UIPAF患者和123例IPAF患者。UIPAF患者在自身免疫特征方面与IPAF患者相似，除了UIPAF患者中类风湿因子的患病率和IPAF患者中抗SSA的患病率。两个队列中进展为特定自身免疫性疾病（SAD）的比例相似，但所发展的SAD类型有所不同。UIPAF患者的实际管理和预后与IPF几乎相同。