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以孤立性吞咽困难为首发症状的PL-7相关免疫介导坏死性肌病1例罕见病例

A Rare Case of PL-7-Associated Immune-Mediated Necrotizing Myopathy With Isolated Dysphagia as the Presenting Symptom.

作者信息

Khan Tahir, Shareef Aleeya, Shahid Mohammad, Shabbir Ehsan, Musleh Mustafa

机构信息

Internal Medicine, Premier Miami Valley Hospital, Dayton, USA.

Internal Medicine, Wright State University Boonshoft School of Medicine, Dayton, USA.

出版信息

Cureus. 2023 Apr 6;15(4):e37215. doi: 10.7759/cureus.37215. eCollection 2023 Apr.

Abstract

Immune-mediated necrotizing myopathy (IMNM) is a rare, progressive disease that accounts for about 19% of all inflammatory myopathies. Dysphagia occurs in about 20%-30% of IMNM patients. This case results in the third presumptive instance of IMNMwith dysphagia as the initial symptom. Given that isolated dysphagia in IMNM is atypical to the conventional symptoms in the late stage of the disease, it is critical for clinicians to have a high degree of suspicion for IMNM due to the aggressive nature of the disease and its refractoriness to treatment. Additionally, this case also highlights an atypical autoantibody, PL-7, being positive in an IMNM patient who presents with dysphagia as an initial symptom.

摘要

免疫介导性坏死性肌病(IMNM)是一种罕见的进行性疾病,约占所有炎性肌病的19%。约20%-30%的IMNM患者会出现吞咽困难。该病例是第三例以吞咽困难为首发症状的疑似IMNM病例。鉴于IMNM中孤立性吞咽困难与该疾病晚期的传统症状不同,由于该疾病具有侵袭性且治疗难治,临床医生对IMNM保持高度怀疑至关重要。此外,该病例还凸显了一种非典型自身抗体PL-7在一名以吞咽困难为首发症状的IMNM患者中呈阳性。

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本文引用的文献

4
Myopathies featuring early or prominent dysphagia.以早发性或显著吞咽困难为特征的肌病。
Muscle Nerve. 2020 Sep;62(3):344-350. doi: 10.1002/mus.26996. Epub 2020 Jun 18.
6
Seronegative patients form a distinctive subgroup of immune-mediated necrotizing myopathy.血清阴性患者构成免疫介导性坏死性肌病的一个独特亚组。
Neurol Neuroimmunol Neuroinflamm. 2018 Oct 16;6(1):e513. doi: 10.1212/NXI.0000000000000513. eCollection 2019 Jan.
7
Immune-Mediated Necrotizing Myopathy.免疫介导性坏死性肌病。
Curr Rheumatol Rep. 2018 Mar 26;20(4):21. doi: 10.1007/s11926-018-0732-6.
8
Syndrome in question: antisynthetase syndrome (anti-PL-7).所讨论的综合征:抗合成酶综合征(抗PL - 7)。
An Bras Dermatol. 2016 Sep-Oct;91(5):683-685. doi: 10.1590/abd1806-4841.20164449.

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