igg4相关的动脉周围炎/腹膜后纤维化和动脉周围炎的最新临床、诊断和治疗进展

IF 4.6 2区医学 Q1 RHEUMATOLOGY

Seminars in arthritis and rheumatism Pub Date : 2025-02-23 DOI:10.1016/j.semarthrit.2025.152691

Mitsuhiro Kawano

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引用次数: 0

摘要

背景：igg4相关疾病（IgG4-RD）是一种全身性、慢性免疫介导的炎症性疾病，与结节病类似，可影响多种器官和组织。igg4相关性主动脉周炎(PAo)/腹膜后纤维化（RPF）是IgG4-RD的五大主要表现之一。尽管在2019年引入了IgG4-RD的ACR和EULAR分类标准，但诊断igg4相关的PAo/RPF和动脉周围炎（PA）仍然具有挑战性，因为很难从这些病变中获得活检。此外，虽然糖皮质激素在治疗IgG4-RD方面非常有效，但处理主动脉或动脉病变却面临着独特的挑战。目的：本文简要回顾了日本对igg4相关PAo/RPF和PA的器官特异性诊断标准的应用，以及与这些病变相关的器官特异性问题的治疗策略的最新进展。方法：首先，我们分析了99例igg4相关PAo/RPF和PA的专家诊断，提出了器官特异性的诊断标准。接下来，我们回顾性分析了另外110例与igg4相关的PAo/RPF和PA患者，以及73例具有临床特征需要与真正的igg4 rd区分的模拟者，以验证所提出的标准。结果：仅33例患者获得组织病理标本（20例主动脉周围病变，5例冠状动脉病变，4例髂动脉病变，1例肠系膜动脉病变，5例不累及动脉的腹膜后病变）。其中71.4%表现为故事状纤维化，71.4%表现为闭塞性静脉炎。所有标本高倍视场平均IgG4阳性浆细胞数均超过10个，32例标本IgG4/ igg阳性细胞比超过40%（91.4%）。血清IgG4水平升高和其他受IgG4- rd影响的器官的特征性受累，对诊断IgG4相关的PAo/RPF和PA至关重要。验证分析证实，结合“心包炎的影像学表现”、“嗜酸性粒细胞浸润或淋巴样卵泡”和“可能的额外pao /PA/RPF病变诊断”，将敏感性从68.4%提高到77.2%，特异性仅略有降低（从97.4%降低到94.7%）。结论：与诊断涉及其他主要器官（如泪腺、唾液腺、胰腺和肾脏）的IgG4-RD相比，即使使用最新的诊断或分类标准，诊断igg4相关的PAo/RPF和PA仍然具有挑战性。此外，在治疗igg4相关PAo/RPF和PA患者时，在制定治疗策略时必须考虑器官特异性因素。

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Update on IgG4-related periaortitis/retroperitoneal fibrosis and periarteritis -recent clinical, diagnostic and therapeutic advances

Background

IgG4-related disease (IgG4-RD) is a systemic, chronic immune-mediated inflammatory disorder that, similar to sarcoidosis, can affect various organs and tissues. IgG4-related periaortitis (PAo)/retroperitoneal fibrosis (RPF) is among the five major manifestations of IgG4-RD. Despite introduction of the ACR and EULAR classification criteria for IgG4-RD in 2019, diagnosing IgG4-related PAo/RPF and periarteritis (PA) remains challenging because obtaining biopsies from these lesions is difficult. Additionally, while glucocorticoids are highly effective in treating IgG4-RD, managing aortic or arterial lesions poses unique challenges.

Objectives

This brief review discusses the utility of Japanese organ-specific diagnostic criteria for IgG4-related PAo/RPF and PA, along with recent advances in treatment strategies including management of organ-specific issues related to these lesions.

Methods

First, we analyzed 99 patients with IgG4-related PAo/RPF and PA based on expert diagnoses to propose organ-specific diagnostic criteria. Next, we retrospectively analyzed an additional 110 patients with IgG4-related PAo/RPF and PA, as well as 73 mimickers with clinical features requiring differentiation from true IgG4-RD to validate the proposed criteria.

Results

Histopathological specimens were obtained from only 33 patients (20 periaortic, 5 coronary arteries, 4 iliac arteries, 1 mesenteric artery, and 5 retroperitoneal lesions not involving arteries). Among these, 71.4 % showed storiform fibrosis, and 71.4 % displayed obliterative phlebitis. The mean number of IgG4-positive plasma cells exceeded 10 per high-power field in all specimens, and the IgG4/IgG-positive cell ratio exceeded 40 % in 32 specimens (91.4 %). Radiographic findings were essential for diagnosing IgG4-related PAo/RPF and PA, supported by elevated serum IgG4 levels and the presence of characteristic involvement of other organs affected by IgG4-RD. Validation analysis confirmed that incorporating “imaging findings of pericarditis”, “eosinophilic infiltration or lymphoid follicles”, and “probable diagnosis of extra-Pao/PA/RPF lesions” improved sensitivity from 68.4 % to 77.2 %, with only a minimal reduction in specificity (from 97.4 % to 94.7 %).

Conclusions

Diagnosing IgG4-related PAo/RPF and PA remains challenging even when using the latest diagnostic or classification criteria, compared to diagnosing IgG4-RD involving other major organs, such as lacrimal and salivary glands, pancreas, and kidneys. In addition, when treating patients with IgG4-related PAo/RPF and PA, organ-specific factors must be considered when developing treatment strategies.

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来源期刊

Seminars in arthritis and rheumatism 医学-风湿病学

CiteScore

9.20

自引率

4.00%

发文量

176

审稿时长

46 days

期刊介绍： Seminars in Arthritis and Rheumatism provides access to the highest-quality clinical, therapeutic and translational research about arthritis, rheumatology and musculoskeletal disorders that affect the joints and connective tissue. Each bimonthly issue includes articles giving you the latest diagnostic criteria, consensus statements, systematic reviews and meta-analyses as well as clinical and translational research studies. Read this journal for the latest groundbreaking research and to gain insights from scientists and clinicians on the management and treatment of musculoskeletal and autoimmune rheumatologic diseases. The journal is of interest to rheumatologists, orthopedic surgeons, internal medicine physicians, immunologists and specialists in bone and mineral metabolism.