晚发型嗜酸性粒细胞增多症伴多发性炎导致肺纤维化:基于病例的综述。

Q4 Medicine
Mediterranean Journal of Rheumatology Pub Date : 2024-02-12 eCollection Date: 2024-03-01 DOI:10.31138/mjr.161023.lpe
Juan Felipe Coronado-Sarmiento, Juan Pablo Coronado-López, Eduardo Tuta-Quintero, Claudia Marcela Mora, Viviana Mayor
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引用次数: 0

摘要

简介嗜酸性粒细胞肉芽肿伴多血管炎(eGPA)是一种中小型血管坏死性血管炎,患者多为四至六十岁,是一种非常罕见的肺纤维化病因:一名 72 岁的西班牙裔女性患者,因下肢疼痛、麻痹、水肿和偶尔出现大面积血尿而就诊。讨论:eGPA 是一种发病率较低的自身免疫性血管炎,表型较多,因此临床表现范围较广,但最近的研究进展有助于了解其生理病理及其与肺纤维化等其他疾病的联系:结论:这种疾病必须及早诊断和治疗,因为这是改变患者预后的唯一因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Late-Presenting Eosinophilic Granulomatosis with Polyangiitis as Cause of Pulmonary Fibrosis: A Case-Based Review.

Introduction: Eosinophilic granulomatosis with polyangiitis (eGPA) is a necrotising vasculitis of small and medium calibre vessels, which affects mostly patients in their fourth to sixth decade of life, and it is a very uncommon aetiology for pulmonary fibrosis.

Clinical case: A Hispanic 72-year-old female patient presents with a history of lower extremities pain, paraesthesia, oedema, and occasional macroscopic haematuria. During her hospitalisation, the patient presents, and images showed findings compatible with pulmonary fibrosis and alveolar haemorrhage, which require a biopsy, establishing the diagnosis of an eGPA.

Discussion: eGPA is a low-incidence autoimmune vasculitis, with a high number of phenotypes which explain the broad clinical spectrum, but recent advances has helped to understand the physiopathology and its link with other conditions like pulmonary fibrosis.

Conclusion: Early diagnosis and management of this condition is mandatory because it is the only factor that change the outcome of the patients.

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CiteScore
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