MPO-ANCA 间质性肺病的表现和进展

IF 4.7 Q2 IMMUNOLOGY
Lorenzo Salvati , Boaz Palterer , Elena Lazzeri , Emanuele Vivarelli , Marina Amendola , Marco Allinovi , Leonardo Caroti , Alessio Mazzoni , Laura Lasagni , Giacomo Emmi , Edoardo Cavigli , Marco Del Carria , Linda Di Pietro , Mariangela Scavone , Daniele Cammelli , Federico Lavorini , Sara Tomassetti , Elisabetta Rosi , Paola Parronchi
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引用次数: 0

摘要

MPO-ANCA相关性血管炎(AAV)与间质性肺病(ILD)之间的联系已经得到证实。肺纤维化可能与 AAV 同时存在,也可能发生在 AAV 诊断之后甚至之前,而肺纤维化的存在会对预后产生不利影响。对 ILD 患者进行 ANCA 检查的最佳方法仍是一个争论不休的话题。在此,我们旨在描述 MPO-ANCA ILD 的表现和进展。我们对2016年6月至2022年6月期间在意大利佛罗伦萨卡雷吉大学医院免疫学和细胞治疗室确诊为MPO-ANCA ILD(伴有或不伴有肾功能损害)的一组患者进行了回顾性评估。研究人员收集了临床病历、影像学检查、病理学检查和实验室检验结果。在 14 例 MPO-ANCA ILD 患者中,我们观察到 ILD 诊断时评估的 MPO-ANCA 滴度与肾脏受累之间存在显著关联。这些病例的肾功能损害通常表现为亚临床或缓慢进展的肾损害。有趣的是,在所有肾活检标本中都发现了补体C3沉积,这表明在控制与MPO-ANCA ILD相关的肾脏并发症方面,有可能找到新的治疗靶点。MPO-ANCA血管炎表现为ILD可能是首次也是唯一的临床表现。ILD诊断时的MPO-ANCA水平可对MPO-ANCA ILD患者肾脏受累的进展情况发出警告,因此需要谨慎,因为肾脏疾病可能是亚临床或隐匿性的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Presentation and progression of MPO-ANCA interstitial lung disease

The association between MPO-ANCA-associated vasculitis (AAV) and interstitial lung disease (ILD) has been well established. Pulmonary fibrosis may coexist with, follow, or even precede the diagnosis of AAV, and its presence adversely affects the prognosis. The optimal approach to investigating ANCA in patients with ILD remains a subject of ongoing debate. Here we aim to describe presentation and progression of MPO-ANCA ILD. We conducted a retrospective evaluation of a cohort of individuals diagnosed with MPO-ANCA ILD, with or without accompanying renal impairment, at the Immunology and Cell Therapy Unit, Careggi University Hospital, Florence, Italy, between June 2016 and June 2022. Clinical records, imaging studies, pathologic examinations, and laboratory test results were collected. Among the 14 patients identified with MPO-ANCA ILD, we observed a significant association between MPO-ANCA titers assessed at the time of ILD diagnosis and renal involvement. Renal impairment in these cases often manifested as subclinical or slowly progressive kidney damage. Interestingly, complement C3 deposits were consistently found in all renal biopsy specimens, thereby suggesting the potential for novel therapeutic targets in managing renal complications associated with MPO-ANCA ILD. The presentation of MPO-ANCA vasculitis as ILD can be the first and only clinical manifestation. MPO-ANCA levels at ILD diagnosis could warn on the progression to renal involvement in patients with MPO-ANCA ILD, hence caution is needed because renal disease can be subclinical or smoldering.

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来源期刊
Journal of Translational Autoimmunity
Journal of Translational Autoimmunity Medicine-Immunology and Allergy
CiteScore
7.80
自引率
2.60%
发文量
33
审稿时长
55 days
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