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探索具有自身免疫特征的间质性肺炎:病例研究见解与当前文献综述

Exploring Interstitial Pneumonia With Autoimmune Features: Case Study Insights and Review of Current Literature.

作者信息

Tajik Jalayeri Mohammad Hadi, Sadani Somayeh, Lashkarbolouk Narges, Mazandarani Mahdi, Razzazan Mehrnaz

机构信息

Clinical Research Development Unit (CRDU), Sayad Shirazi Hospital Golestan University of Medical Sciences Gorgan Iran.

Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute Tehran University of Medical Sciences Tehran Iran.

出版信息

Clin Case Rep. 2025 Aug 29;13(9):e70828. doi: 10.1002/ccr3.70828. eCollection 2025 Sep.

Abstract

Interstitial Pneumonia with Autoimmune Features (IPAF) refers to a subgroup of interstitial lung diseases that show autoimmune-like characteristics but do not align with established conditions such as idiopathic pulmonary fibrosis (IPF) or recognized connective tissue diseases (CTD). In this case report, we present a 43-year-old woman who had a history of dry cough, decreased appetite, weight loss, and progressive shortness of breath. Initial investigations, including spirometry, autoantibody testing, and CT scan, revealed evidence of nonspecific interstitial pneumonia and positive autoantibodies. Our patient did not fully meet the CTD criteria, and based on the clinical symptoms, imaging findings, and the presence of autoantibodies, an IPAF diagnosis was made. Our patient was treated with prednisolone and mycophenolate, significantly improving her symptoms, pulmonary function, and exercise capacity during the one-year follow-up period. Our report mentioned the importance of considering IPAF in the differential diagnosis of patients presenting with interstitial lung disease and features of an autoimmune process. Delayed diagnosis contributes to poorer prognoses and increased disease severity among affected individuals.

摘要

具有自身免疫特征的间质性肺炎(IPAF)是指间质性肺疾病的一个亚组,其表现出类似自身免疫的特征,但不符合特发性肺纤维化(IPF)或公认的结缔组织病(CTD)等既定疾病。在本病例报告中,我们介绍了一名43岁女性,她有干咳、食欲减退、体重减轻和进行性气短病史。包括肺功能测定、自身抗体检测和CT扫描在内的初步检查显示有非特异性间质性肺炎的证据和自身抗体阳性。我们的患者不完全符合CTD标准,基于临床症状、影像学表现和自身抗体的存在,做出了IPAF诊断。我们的患者接受了泼尼松龙和霉酚酸酯治疗,在一年的随访期内症状、肺功能和运动能力显著改善。我们的报告提到了在对出现间质性肺疾病和自身免疫过程特征的患者进行鉴别诊断时考虑IPAF的重要性。延迟诊断会导致受影响个体的预后较差和疾病严重程度增加。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/304d/12396933/aaa297afff62/CCR3-13-e70828-g003.jpg

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