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Hindawi Case Reports in Immunology Volume 2022, Article ID 8541329, 5 pages https://doi.org/10.1155/2022/8541329 Case Report Systemic Sclerosis, Reversible Cerebral Vasoconstriction Syndrome, and NeuroMyelitis Optica in a Patient 1 2 3 2 Masoud Etemadifar , Mehdi Shaﬁei , Mehri Salari , Ali Modares Sadeghi , and Mohammad Fakhrolmobasheri Department of Neurology, Isfahan University of Medical Sciences, Isfahan, Iran Department of Neurosurgery, Isfahan University of Medical Sciences, Isfahan, Iran Functional Neurosurgery Research Center, Shohada Tajrish Comprehensive Neurosurgical Center of Excellence, Shahid Beheshti University of Medical Sciences, Tehran, Iran Isfahan Cardiovascular Research Center (Heart Failure Research Center), Isfahan, Iran Correspondence should be addressed to Mohammad Fakhrolmobasheri; email@example.com Received 1 April 2022; Revised 12 June 2022; Accepted 1 July 2022; Published 12 July 2022 Academic Editor: Ahmad Mansour Copyright © 2022 Masoud Etemadifar et al. �is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. 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. �is 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 modi”ed regarding the risk of scleroderma renal crisis. RCVS is a neuro-vascular disorder characterized by reversible 1. Introduction spasm of cerebral arteries . �e exact pathophysiology of Systemic sclerosis (SSC) is a connective tissue disorder with RCVS is not clearly de”ned; however, several risk factors are organ speci”c manifestations limited to the skin or systemic discovered including the administration of high dose glu- manifestations including lungs, kidneys, and gastrointestinal cocorticoid . RCVS may contribute to subarachnoid  and intracerebral hemorrhages  which may contribute to system involvement. NMO is characterized by excessive stromal collagen deposition and autoimmunity caused by major morbidity for patients. �e incidence of RCVS in the autoantibodies which are mostly reported to be anti-topo- context of autoimmune disorders is rarely reported [7, 8]. isomerase, anti-scl70, and anti-nuclear antibody. Microvas- Renal crisis is a lethal complication of systemic sclerosis cular complications in SSC may manifest as Raynaud manifesting with malignant hypertension and acute renal phenomena, renal crisis, and pulmonary hypertension . failure. �is condition may occur in patients treated by high Raynaud phenomena could also appear in the brain causing dose glucocorticoids and could pose permanent damage to reversible cerebral vasoconstriction syndrome (RCVS) . the kidneys . 2 Case Reports in Immunology Neuromyelitis optica (NMO) is an autoimmune disease coarse speckeld pattern, anti-nRNP/sm � 92 (strong positive of the CNS mostly involving the optic nerves, spinal cord, >50), anti-sm � 8 (6< borderline< 10), anti-nucleosomes antibody � 20 (11< positive+< 25), anti-histones � 20 and brainstem mainly manifesting with blurred vision, acute onset paralysis, intractable nausea, vomiting, and hiccups. (11< positive+< 25), and anti-cyclic citrollinated peptide Antibodies against aquapourin-4, the prime channel regu- antibody � 29.3 (positive >18). &e diagnosis was made lating cellular hemostasis in the CNS, are the main anti- according to ACR/EULAR criteria for classiﬁcation of sys- bodies discovered in association with NMO (NMO-IgG) temic sclerosis with a score of 15 (skin thickening � 9, ab- . &e autoantibodies induce necroptosis in myelin normal nailfold capillaries � 2, Raynaud phenomenon � 3, producing cell manifesting as inﬂammatory demyelination laboratory ﬁndings � 1) . &e patient was treated with of the involved region in the CNS. &e relapsing behavior of calcium channel blockers, proton pump inhibitors, and ﬂu- NMO may eventually lead to extreme morbidities for the oxetine. For about one and a half years, her clinical status was patient whereas severe optic neuritis may lead to blindness, stable. About 6 months later, she experienced blurred vision in longitudinal extensive transverse myelitis (LETM) may lead her left eye. MRI of the brain was normal; however, optic perimetry indicated an altitudinal ﬁeld defect in the left. to paralysis, and brainstem involvements could end in acute central respiratory failure . Ophthalmology consultation proposed the diagnosis of optic As any other autoimmune diseases, NMO is reported in neuritis in association with systemic sclerosis. &us, a meth- association with many systemic autoimmunities such as ylprednisolone pulse was administered in four consecutive systemic lupus erythematous, sarcoidosis, and Sjogren’s ¨ doses of 1 gram daily under close observation. 24 hours after syndrome. NMO is also associated with organ speciﬁc au- administration of the last dose, she experienced a sudden onset toimmune diseases such as autoimmune thyroiditis, my- of thunderclap headache, nausea, vomiting, and loss of con- asthenia gravis, idiopathic thrombocytopenic purpura, and sciousness. Early assessments revealed a blood pressure of 220/ ulcerative colitis. However, the coexistence of NMO and 190, GCS of 7/15, reactive and symmetric pupils, no gaze, and systemic sclerosis and further complications in treatment is positive corneal and gag reﬂex. Brain computed tomography rarely reported [10, 12]. On the other hand, the occurrence (CT) implied huge intracranial hemorrhage in the left pari- etotemporal region. Cerebral arteries angiography showed of RCVS in the context of SSC is explainable through similar mechanisms but is diﬃcult to correlate. severe vasospasm in both internal carotid arteries at c4 and c6 Herein, we present a patient with systemic sclerosis who segments and left middle cerebral artery’s M1 which is con- later developed optic neuritis. She was treated for optic sistent with reversible cerebrovascular spasm (RCVS). Even- neuritis with glucocorticoids which targeted scleroderma tually, considering very high intracranial pressure, she renal crisis and RCVS. &e RCVS further caused SAH. underwent an extensive craniotomy for brain decompression, Regarding increased ICP, she underwent a craniotomy and after a month of hospitalization, she was discharged with surgery. Two months later, after resolution of increased ICP, GCS � 15, left hemiplegia, and dysarthria. After 4 months of carnioplasty was performed for her. One day after discharge observation, the patient’s status became eligible for cranio- from the neurosurgery ward, she presented again with plasty. She underwent the surgery and after 4 days of hos- paraplegia and urinary retention. Further investigations pitalization, she was discharged with a favorable clinical status. revealed LETM which was consistent with NMO. 24 hours later, on February 18, 2021, she was readmitted to our clinic with complaints of new onset paraplegia and urinary retention which could have been easily misdiagnosed 2. Case Report for postoperative complications. On the contrary, physical examinations demonstrated bilateral positive Babinski sign A 35-year-old female referred to our clinic complaining of paraplegia and urinary retention one day after discharge from and a sensory level at C4 which led us to suspect a new onset the hospital following a carnioplasty surgery. Her medical spinal disorder rather than any adverse incidence following history revealed that 2 years ago she had been diagnosed with the cranioplasty. &erefore, we performed a magnetic reso- systemic sclerosis manifested as intractable nausea and nance imaging (MRI) of the spinal cord, and the results showed a longitudinally extensive transverse myelitis (LETM) vomiting. At that time, routine workups had been insigniﬁcant except for a history of chronic fatigue and hypothyroidism. from C4 to conus medullaris (Figure 1). &e blood test results indicated high titer of anti-aquaporin4 antibody (1 : 320) &e hypothyroidism was diagnosed 1 year before the diagnosis of SSC. &e patient’s records indicated that the thyroid dys- which conﬁrmed the diagnosis of NMO. Consequently, she underwent plasmapheresis (for 7 days, 250 ml/kg) without function was diagnosed to be due to Hashimoto thyroiditis with positive anti-thyroid peroxidase antibodies (anti-TPO). methylprednisolone. Following the treatment, her clinical status started to improve and the new onset paralysis in her Physical examination was indicative for cutaneous manifes- tations of scleroderma; Rodnan score 2 (puﬀy hands and shiny left leg gradually diminished. After 9 days of hospitalization, skin with skin thickening and a dull face). In addition, high the patient was discharged with a favorable clinical status and erythrocyte sedimentation rate (ESR) in laboratory studies and the ability to walk with minor assistance. a grade II esophagitis in upper GI endoscopy was noted in her medical records. After a comprehensive rheumatologic 2.1. Ethical Statement. &is study was approved by com- workup, the diagnosis of systemic sclerosis was eventually mittee of research, neurosurgery department, Isfahan Uni- conﬁrmed. &e laboratory records indicated anti-nuclear versity of Medical Sciences, Iran. Written informed consent antibody � 1/3200 (positive >1 :100) with a nuclear large/ Case Reports in Immunology 3 Figure 1: STIR view in magnetic resonance imaging of the patient’s spinal cord indicates longitudinally extensive transverse myelitis. was obtained from the patient. &e consent form is available autoimmunity and SSC is extensively studied. &e authors on reasonable request to the corresponding author. postulated that T-helper 1 immune predominance in combination with vitamin D deﬁciency may be the same triggers for autoimmune thyroid disorders and SSC . 3. Discussion and Conclusion Nevertheless, the coexistence of thyroid autoimmunity, SSC and NMO in the present case is novel in the literature and Systemic sclerosis is rarely reported in association with NMO. According to the study by Bollo et al., till now, there may be a clue for further researches in this ﬁeld whereas the treatment of such conditions may become challenging due to has been only seven cases reported to be suﬀering from concomitant NMO and SSC in which all of them were the fact that glucocorticoid treatment in SSC patients may treated by glucocorticoids . &e time of onset of SSC and trigger the development of renal crisis. Renal crisis may NMO diﬀered among cases as in the case reported by manifest as hypertension and acute renal injury. Although Hernandez et al. NMO was developed years after the di- the condition satisfactorily responds to treatment with agnosis of SSC and in the case studied by Moriguchi et al. angiotensin converting enzyme inhibitors (ACEI), severe NMO was diagnosed one year before the symptoms of SSC. complications such as malignant hypertension, intracranial Interestingly, all cases were female with age from 30 to 65 hemorrhage, and permanent renal impairment could still years. None of the reported cases had concomitant thyroid ensue . In the case reported by Deeb et al. , the patient developed systemic sclerosis renal crisis following the ad- disorder except in our case who was diagnosed for hypo- thyroidism about 3 years before the diagnosis of NMO. ministration of glucocorticoid; however, other cases of &yroid disorders such as Hashimoto thyroiditis may ac- which all were treated with high dose methylprednisolone company NMO. Shahmohammadi et al. had postulated that did not develop renal crisis [12, 14, 18–21]. Accordingly, in autoimmune thyroiditis, Hashimoto thyroiditis, and graves’ the present case, intracerebral and subarachnoid hemor- disease are the most common organ speciﬁc autoimmune rhage after administration of glucocorticoids was notable in disorders associated with NMO. &e presence of antithyroid her medical history. antibodies is frequently reported in patients with NMO. Another interesting ﬁnding from the studies is that some Moreover, it has been reported that thyroid follicular cells cases did not respond well to the treatment with steroids and may have AQP4 containing channels . In another review the patients were subsequently treated with other immu- by Fallahi et al., the association between thyroid nosuppressive agents such as cyclophosphamide . &is 4 Case Reports in Immunology Emergency Medicine International, vol. 2012, Article ID might highlight the importance of choosing proper treat- e303152, 2012 pages, 2012. ment for patients with such complicated condition. 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Case Reports in Immunology – Hindawi Publishing Corporation
Published: Jul 12, 2022
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