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自身免疫性血管炎中的蛛网膜下腔出血:系统性红斑狼疮-抗中性粒细胞胞浆抗体相关血管炎重叠综合征的一种罕见表现。

Subarachnoid Hemorrhage in Autoimmune Vasculitis: A Rare Presentation of Systemic Lupus Erythematosus-Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis Overlap Syndrome.

作者信息

Palleti Sujith K, Larson Hanna, Avula Sreekant, Picken Maria M, Wadhwa Anuradha

机构信息

Nephrology, Edward Hines Jr. Veterans Administration Hospital, Hines, USA.

Nephrology, Loyola University Medical Center, Maywood, USA.

出版信息

Cureus. 2023 May 3;15(5):e38482. doi: 10.7759/cureus.38482. eCollection 2023 May.

Abstract

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and systemic lupus erythematosus (SLE), though they share similar clinical characteristics, are distinguishable based on specific characteristics. The concomitant presentation of SLE and AAV as overlap syndrome is rare and makes the diagnosis challenging. Here, we describe a rare case of SLE and AAV overlaps presenting with hemorrhagic stroke as initial presentation, which has been reported only once before. The presence of several positive autoantibodies made the diagnosis challenging, but a kidney biopsy provided the definitive diagnosis and aided in initiating immunosuppressive therapy. The patient did not respond to standard initial surgical measures to lower elevated intracranial pressure and showed significant improvement to immunosuppressive therapy proving the temporal relationship. The authors of this case study aim to highlight the importance of considering SLE-AAV overlap in patients presenting with features similar to those described in the case report and intervening early, as delays in diagnosis can be fatal.

摘要

抗中性粒细胞胞浆自身抗体(ANCA)相关性血管炎(AAV)和系统性红斑狼疮(SLE)虽然具有相似的临床特征,但可根据特定特征加以区分。SLE和AAV以重叠综合征的形式同时出现较为罕见,这使得诊断具有挑战性。在此,我们描述了一例罕见的SLE与AAV重叠病例,其首发表现为出血性中风,此前仅有一次相关报道。多种自身抗体呈阳性使得诊断颇具挑战,但肾脏活检提供了明确诊断,并有助于启动免疫抑制治疗。患者对降低颅内压升高的标准初始手术措施无反应,而对免疫抑制治疗有显著改善,证明了两者的时间关系。本病例研究的作者旨在强调,对于表现出与病例报告中所述特征相似的患者,考虑SLE-AAV重叠并尽早干预的重要性,因为诊断延迟可能是致命的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e127/10238284/ca42c3ac0418/cureus-0015-00000038482-i01.jpg

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