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嗜酸性粒细胞性肺炎与干燥综合征的令人困惑的共存:诊断困境

The Puzzling Coexistence of Eosinophilic Pneumonia With Sjogren's Syndrome: A Diagnostic Dilemma.

作者信息

Liang Jessica, Shapoo Mazhar, Rasendrakumar Arabi

机构信息

Internal Medicine, Wayne State University Detroit Medical Center, Detroit, USA.

Rheumatology, Wayne State University Detroit Medical Center, Detroit, USA.

出版信息

Cureus. 2024 Jul 13;16(7):e64470. doi: 10.7759/cureus.64470. eCollection 2024 Jul.

DOI:10.7759/cureus.64470
PMID:39135819
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11318719/
Abstract

We present a case where a patient with no significant pulmonary nor autoimmune medical history presents with acute hypoxic respiratory failure and a dry cough that's made worse when conversing. She gets diagnosed with eosinophilic pneumonia after bronchoalveolar lavage (BAL) showed 70% eosinophils while also having labs highly suggestive of primary Sjogren's syndrome (pSS) with an anti-SSA titer of 111.3 U/mL and anti-SSA 52 kD Ab, immunoglobulin (Ig)G >200 U. The initial treatment plan was to start rituximab to target primary Sjogren's syndrome associated interstitial lung disease (pSS-ILD), however after close discussion with pulmonology, it was changed to mepolizumab to target eosinophilic pneumonia. From a diagnostic standpoint, it may be tricky to determine which disease process is driving the symptoms especially when the patient has labs that are convincing for both.

摘要

我们呈现这样一个病例

一名既往无显著肺部疾病及自身免疫性疾病病史的患者出现急性低氧性呼吸衰竭,伴有干咳,交谈时加重。支气管肺泡灌洗(BAL)显示嗜酸性粒细胞占70%,同时实验室检查高度提示原发性干燥综合征(pSS),抗SSA滴度为111.3 U/mL,抗SSA 52 kD抗体,免疫球蛋白(Ig)G>200 U,该患者被诊断为嗜酸性粒细胞性肺炎。最初的治疗方案是开始使用利妥昔单抗治疗原发性干燥综合征相关间质性肺病(pSS-ILD),然而,在与肺病科密切讨论后,改为使用美泊利单抗治疗嗜酸性粒细胞性肺炎。从诊断的角度来看,确定是哪种疾病过程导致症状可能很棘手,尤其是当患者的实验室检查结果对两种疾病都有说服力时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9629/11318719/8c1d952c8ea0/cureus-0016-00000064470-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9629/11318719/8c1d952c8ea0/cureus-0016-00000064470-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9629/11318719/8c1d952c8ea0/cureus-0016-00000064470-i02.jpg

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本文引用的文献

1
Characteristics and mortality in primary Sjögren syndrome-related interstitial lung disease.原发性干燥综合征相关间质性肺病的特征和死亡率。
Medicine (Baltimore). 2021 Sep 3;100(35):e26777. doi: 10.1097/MD.0000000000026777.
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Interstitial lung disease in non-sicca onset primary Sjögren's syndrome: a large-scale case-control study.非干燥起病的原发性干燥综合征中的间质性肺疾病:一项大规模病例对照研究
Int J Rheum Dis. 2018 Jul;21(7):1423-1429. doi: 10.1111/1756-185X.13334.
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Prognostic factors in interstitial lung disease associated with primary Sjögren's syndrome: a retrospective analysis of 33 pathologically-proven cases.
原发性干燥综合征相关间质性肺病的预后因素:33 例经病理证实病例的回顾性分析。
PLoS One. 2013 Sep 9;8(9):e73774. doi: 10.1371/journal.pone.0073774. eCollection 2013.
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