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埃文斯综合征合并类风湿关节炎和系统性红斑狼疮:自身免疫交叉的一个独特病例。

Evans syndrome in conjunction with rheumatoid arthritis and SLE: A unique case of autoimmune intersection.

作者信息

Hashim Hashim Talib, Al-Obaidi Ahmed Dheyaa, Al-Fatlawi Nabeel, Hassan Karrar Hussein, Al-Asadi Azhar Kareem, Al-Rihaymee Afrah Majeed Ahmed, Al-Obiade Reem, Joffrey Meelad, Al-Badri Sajjad Ghanim, Al-Awad Abdullah

机构信息

Research Department, University of Warith Al-Anbiyaa, College of Medicine, Karbala, Iraq.

Deparment of Internal Medicine, University of Baghdad, College of Medicine, Baghdad, Iraq.

出版信息

Radiol Case Rep. 2024 Sep 25;19(12):6323-6327. doi: 10.1016/j.radcr.2024.09.053. eCollection 2024 Dec.

DOI:10.1016/j.radcr.2024.09.053
PMID:39387031
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11461976/
Abstract

Evans syndrome is a rare autoimmune disorder characterized by autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), often linked with systemic lupus erythematosus (SLE). We present a case of a 25-year-old female with a history of rheumatoid arthritis (RA) who presented with new SLE symptoms, including left-sided weakness, pallor, and a photosensitive rash. Laboratory tests confirmed Evans syndrome, and MRI showed a cerebral infarction. Treatment with corticosteroids, hydroxychloroquine, and mycophenolate mofetil led to significant improvement. This case highlights the complexity of managing Evans syndrome in patients with coexisting autoimmune diseases like RA and SLE, emphasizing the need for early and aggressive treatment.

摘要

伊文氏综合征是一种罕见的自身免疫性疾病,其特征为自身免疫性溶血性贫血(AIHA)和免疫性血小板减少症(ITP),常与系统性红斑狼疮(SLE)相关。我们报告一例25岁女性,有类风湿关节炎(RA)病史,出现了新的SLE症状,包括左侧肢体无力、面色苍白和光敏性皮疹。实验室检查确诊为伊文氏综合征,MRI显示有脑梗死。使用皮质类固醇、羟氯喹和霉酚酸酯进行治疗后有显著改善。该病例凸显了在患有RA和SLE等共存自身免疫性疾病的患者中管理伊文氏综合征的复杂性,强调了早期积极治疗的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db30/11461976/fe1dce244466/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db30/11461976/1a47e90e12f6/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db30/11461976/fc1289e69be2/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db30/11461976/fe1dce244466/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db30/11461976/1a47e90e12f6/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db30/11461976/fc1289e69be2/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db30/11461976/fe1dce244466/gr3.jpg

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本文引用的文献

1
Haploinsufficiency in PTPN2 leads to early-onset systemic autoimmunity from Evans syndrome to lupus.PTPN2 杂合性缺失导致 Evans 综合征到狼疮的早发性系统性自身免疫。
J Exp Med. 2024 Sep 2;221(9). doi: 10.1084/jem.20232337. Epub 2024 Jul 19.
2
A novel overlap syndrome: Rheumatoid arthritis, Sjogren's syndrome, antiphospholipid syndrome, and dermatomyositis.一种新型重叠综合征:类风湿关节炎、干燥综合征、抗磷脂综合征和皮肌炎。
Clin Case Rep. 2023 Apr 24;11(4):e7274. doi: 10.1002/ccr3.7274. eCollection 2023 Apr.
3
Unusual presentation of rheumatoid arthritis in a 106-year-old woman: A rare case report.
106岁女性类风湿关节炎的不寻常表现:一例罕见病例报告。
Clin Case Rep. 2023 Mar 17;11(3):e7120. doi: 10.1002/ccr3.7120. eCollection 2023 Mar.
4
Evans Syndrome in the Context of Incomplete Systemic Lupus Erythematosus.不完全系统性红斑狼疮背景下的伊文氏综合征
Cureus. 2022 Jun 9;14(6):e25795. doi: 10.7759/cureus.25795. eCollection 2022 Jun.
5
New-onset Evans syndrome associated with systemic lupus erythematosus after BNT162b2 mRNA COVID-19 vaccination.接种 BNT162b2 mRNA COVID-19 疫苗后出现与系统性红斑狼疮相关的新发性 Evans 综合征。
Int J Hematol. 2022 Mar;115(3):424-427. doi: 10.1007/s12185-021-03243-2. Epub 2021 Oct 23.
6
Evans syndrome and its link with systemic lupus erythematosus.伊文氏综合征及其与系统性红斑狼疮的关联。
Saudi J Kidney Dis Transpl. 2016 Jan;27(1):147-9. doi: 10.4103/1319-2442.174177.
7
How I manage Evans Syndrome and AIHA cases in children.我是如何管理儿童 Evans 综合征和自身免疫性溶血性贫血病例的。
Br J Haematol. 2016 Feb;172(4):524-34. doi: 10.1111/bjh.13866. Epub 2015 Dec 2.
8
Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome. Evans 综合征与系统性红斑狼疮:临床表现与转归。
Joint Bone Spine. 2012 Jul;79(4):362-4. doi: 10.1016/j.jbspin.2011.07.004. Epub 2011 Sep 23.
9
Evans syndrome: a study of six cases with review of literature.伊文斯综合征:6例病例研究及文献复习
Hematology. 2008 Dec;13(6):356-60. doi: 10.1179/102453308X343518.
10
Acquired hemolytic anemia; the relation of erythrocyte antibody production to activity of the disease; the significance of thrombocytopenia and leukopenia.获得性溶血性贫血;红细胞抗体产生与疾病活动的关系;血小板减少症和白细胞减少症的意义。
Blood. 1949 Nov;4(11):1196-1213.