Systemic Sclerosis, Reversible Cerebral Vasoconstriction Syndrome, and NeuroMyelitis Optica in a Patient.

IF 0.7 Q4 IMMUNOLOGY
Case Reports in Immunology Pub Date : 2022-07-12 eCollection Date: 2022-01-01 DOI:10.1155/2022/8541329
Masoud Etemadifar, Mehdi Shafiei, Mehri Salari, Ali Modares Sadeghi, Mohammad Fakhrolmobasheri
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引用次数: 1

Abstract

Systemic sclerosis (SSC) is an autoimmune disease of connective tissue and microvasculature mostly caused by autoantibodies. Likewise, neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system correlating with autoantibodies against aquapourin-4. Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder of brain vasculature resembling Raynaud phenomena in SSC. Despite co-occurrence is not rare in autoimmune disorders, the co-occurrence of NMO and SSC is extremely rare. In this case, we report a 35-year-old female presenting with paraplegia one day after discharge from hospital following surgical carnioplasty. She had a history of scleroderma and optic neuritis for which she was treated with high dose glucocorticoids causing renal crisis and RCVS causing intracranial and intracerebral hemorrhage which required a craniotomy to be performed in February 2020. In her recent admission, magnetic resonance imaging of the spinal cord indicated longitudinally extensive transverse myelitis (LETM) and blood tests revealed a highly positive titer of NMO-IgG. Daily plasmapheresis resulted in satisfactory improvement in her condition. This case highlights the importance of evaluating neurologic manifestations in systemic sclerosis patients considering the NMO and RCVS occurrence. Additionally, in concomitant cases, the treatment strategy should be modified regarding the risk of scleroderma renal crisis.

Abstract Image

系统性硬化症,可逆性脑血管收缩综合征和视神经脊髓炎患者。
系统性硬化症(SSC)是一种主要由自身抗体引起的结缔组织和微血管自身免疫性疾病。同样,视神经脊髓炎(NMO)是一种中枢神经系统脱髓鞘疾病,与抗水蛋白-4的自身抗体相关。可逆性脑血管收缩综合征(RCVS)是一种类似于SSC中的雷诺现象的脑血管疾病。尽管在自身免疫性疾病中同时发生并不罕见,但NMO和SSC同时发生是极其罕见的。在这个病例中,我们报告了一位35岁的女性,在手术成形术后出院一天后出现截瘫。她有硬皮病和视神经炎病史,曾接受高剂量糖皮质激素治疗,导致肾危象和RCVS,导致颅内和脑出血,需要于2020年2月进行开颅手术。在她最近入院时,脊髓磁共振成像显示纵向广泛横贯脊髓炎(LETM),血液检查显示NMO-IgG高阳性滴度。每日血浆置换使她的病情得到令人满意的改善。考虑到NMO和RCVS的发生,本病例强调了评估系统性硬化症患者神经系统表现的重要性。此外,在合并病例中,应根据硬皮病肾危象的风险修改治疗策略。
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来源期刊
CiteScore
1.90
自引率
0.00%
发文量
14
审稿时长
15 weeks
期刊介绍: Case Reports in Immunology is a peer-reviewed, Open Access journal that publishes case reports and case series related to allergies, immunodeficiencies, autoimmune diseases, immune disorders, cancer immunology and transplantation immunology.
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