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狼疮性脑炎作为系统性红斑狼疮一种罕见的神经精神表现

Lupus Cerebritis as a Rare Neuropsychiatric Manifestation of Systemic Lupus Erythematosus.

作者信息

Leitao Ayola R, Jain Molly S, Luvsannyam Enkhmaa, Jayswal Vanita, Tiesenga Frederick

机构信息

Internal Medicine, Saint James School of Medicine, Park Ridge, USA.

Medicine, Saint James School of Medicine, Park Ridge, USA.

出版信息

Cureus. 2022 May 13;14(5):e24973. doi: 10.7759/cureus.24973. eCollection 2022 May.

Abstract

Mixed connective tissue disease (MCTD) is a rare autoimmune condition that shows an overlap of at least two connective tissue diseases (CTD) including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), scleroderma, Sjögren's syndrome, polymyositis, or dermatomyositis. From a laboratory standpoint, MCTD is associated with high titers of anti-U1-RNP antibodies, which makes it difficult to determine whether it is a variant of each of the respective CTDs or a different entity altogether. Our objective is to report the case of a patient with MCTD presenting with status epilepticus who was ultimately diagnosed with lupus cerebritis. The case also highlights the development of complications unrelated to MCTD that made the management even more challenging. Overall, the authors emphasize the rareness of lupus cerebritis as a presentation, the diagnostic challenges faced due to the lack of classical manifestations of SLE, and how the complicated clinical course makes a downhill prognosis more likely.

摘要

混合性结缔组织病(MCTD)是一种罕见的自身免疫性疾病,表现为至少两种结缔组织病(CTD)重叠,包括系统性红斑狼疮(SLE)、类风湿关节炎(RA)、硬皮病、干燥综合征、多发性肌炎或皮肌炎。从实验室角度来看,MCTD与高滴度的抗U1-RNP抗体相关,这使得难以确定它是各自CTD的一种变体还是完全不同的实体。我们的目的是报告一例患有MCTD且出现癫痫持续状态的患者,该患者最终被诊断为狼疮性脑病。该病例还突出了与MCTD无关的并发症的发生,这使得治疗更具挑战性。总体而言,作者强调狼疮性脑病作为一种表现的罕见性、由于缺乏SLE的典型表现而面临的诊断挑战,以及复杂的临床病程如何更有可能导致预后不良。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/84c0/9188815/f9459552687f/cureus-0014-00000024973-i01.jpg

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