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嗜酸性肉芽肿性多血管炎(EGPA):一例非典型表现的病例报告。

Eosinophilic Granulomatosis with Polyangiitis (EGPA): A case report with atypical presentation.

作者信息

Mahmood Khalid, Butt Nauman Ismat, Ashfaq Fahmina, Aftab Sabeen

机构信息

Khalid Mahmood, MBBS, FCPS. Assistant Professor, Department of Medicine & Allied, Azra Naheed Medial College, Superior University, Lahore, Pakistan.

Nauman Ismat Butt, MBBS, FCPS. Assistant Professor, Department of Medicine & Allied, Azra Naheed Medial College, Superior University, Lahore, Pakistan.

出版信息

Pak J Med Sci. 2023 Jan-Feb;39(1):307-309. doi: 10.12669/pjms.39.1.6436.

DOI:10.12669/pjms.39.1.6436
PMID:36694733
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9842979/
Abstract

A 72-year-old asthmatic gentleman with a history of recurrent sinusitis and chronic bronchitis presented with shortness of breath and progressively worsening hypoxemic respiratory failure. His CT chest demonstrated airspace disease bilaterally with ground-glass opacifications. He had peripheral eosinophilia with raised inflammatory markers but negative work up of infection. On further investigation, ANA was positive, titer 1:160, speckled pattern and both pANCA and cANCA were present. The patient was diagnosed with Eosinophilic Granulomatosis with Polyangiitis (EGPA) and started on intravenous steroids and cyclophosphamide. A rare multi-organ vasculitis, EGPA is hallmarked by asthma, sinusitis and eosinophilia. In initial stages vasculitic involvement is not usually seen thereby making EGPA a diagnostic challenge.

摘要

一位72岁的哮喘男性患者,有复发性鼻窦炎和慢性支气管炎病史,出现呼吸急促和进行性加重的低氧性呼吸衰竭。他的胸部CT显示双侧气腔病变伴磨玻璃影。他有外周血嗜酸性粒细胞增多及炎症指标升高,但感染相关检查结果为阴性。进一步检查发现,抗核抗体(ANA)阳性,滴度为1:160,呈斑点状模式,且同时存在抗蛋白酶3抗体(pANCA)和抗髓过氧化物酶抗体(cANCA)。该患者被诊断为嗜酸性肉芽肿性多血管炎(EGPA),并开始接受静脉注射类固醇和环磷酰胺治疗。EGPA是一种罕见的多器官血管炎,其特征为哮喘、鼻窦炎和嗜酸性粒细胞增多。在疾病初期通常看不到血管炎累及,因此EGPA是一个诊断难题。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54ec/9842979/ad22e7f6b2f9/PJMS-39-307-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54ec/9842979/0cdac1e19c67/PJMS-39-307-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54ec/9842979/ad22e7f6b2f9/PJMS-39-307-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54ec/9842979/0cdac1e19c67/PJMS-39-307-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/54ec/9842979/ad22e7f6b2f9/PJMS-39-307-g002.jpg

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