Aggressive CNS lupus vasculitis in the absence of systemic disease activity

Everett, C M; Graves, Tracey D.; Lad, Shilpa; Jäger, Hans Rolf; Thom, Maria; Isenberg, David; Hanna, Michael G.

doi:10.1093/rheumatology/kem264

Cited by 14 publications

(8 citation statements)

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“…However, with regard to the presenting feature of headache, confusion, seizure, visual disturbance, and psychiatric manifestation in terms of psychosis along with the key fact of there being a lack of clinical improvement in the chief findings mentioned above, raised the possibility of CNS manifestation of SLE rather than an infectious etiology. Furthermore, the suspicion was increased considering there was an insignificant response to the already initiated antimicrobial medication although the other systemic manifestations of SLE were not apparent at that time [7].…”

Section: Discussionmentioning

confidence: 99%

Neuropsychiatric Lupus: A Challenging Journey of a Patient With Pulmonary Tuberculosis

Chapra¹,

Khir²,

Alamami³

et al. 2021

Cureus

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Systemic lupus erythematosus (SLE) is a disease that affects multiple systems in the body. Due to its variable manifestations, it can at times pose challenges for physicians to hold SLE as the culprit behind an affected system. This is most true when encountering patients with neuropsychiatric manifestations of SLE. We present a case of a 38-year-old female with known SLE limited to skin involvement and on treatment for active pulmonary tuberculosis (TB), yet otherwise healthy, who presented with acute fever associated with generalized tonic-clonic seizures. She was investigated for meningoencephalitis with a cerebrospinal fluid (CSF) analysis not being fully conclusive and with imaging features suggestive of viral encephalitis. However, despite receiving optimal care for causes of bacterial, viral, and tuberculous meningitis the patient continued to deteriorate and started to develop predominant psychiatric symptoms in the form of confusion and combative behavior requiring pharmacological restraint. Hence a trial of immunosuppressives was given with a presumptive diagnosis of neuropsychiatric lupus with IV methylprednisolone followed by a course of IV cyclophosphamide. However, this treatment proceeded with caution due to the fear of disseminated tuberculosis for which she did not show any sign of in the subsequent weeks. The patient showed modest clinical and radiological improvement and hence the treatment was continued. The case highlights the uncertainty that may precede a diagnosis of neuropsychiatric lupus and the challenges in treating it in patients with active mycobacterial infection.

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Section: Discussionmentioning

confidence: 99%

Neuropsychiatric Lupus: A Challenging Journey of a Patient With Pulmonary Tuberculosis

Chapra¹,

Khir²,

Alamami³

et al. 2021

Cureus

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“…The American College of Rheumatology recognizes 19 SLE neuropsychiatric syndromes including psychosis, stroke, seizures and subtle abnormalities of cognitive function [6].…”

Section: Discussionmentioning

confidence: 99%

Fulminant neurologic manifestation of Systemic Lupus Erythematosus

et al. 2014

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Central nervous system (CNS) presentations of Systemic Lupus Erythematosus (SLE) may be focal neurological and diffuse psychiatric manifestations. We are reporting a clinical case of severe neurologic manifestations on a 19 year old woman with SLE diagnosed in the Neurology clinic. Severe neurological involvement in SLE is one of the most dreadful complications of the disease, associated with a poor prognosis. The problem remains what is the molecular support of such an important cerebral event. Blood brain barrier endothelium is apparently a target for several pathogenic mechanisms that can account for the CNS manifestations of SLE. The autoimmune response in SLE might affect endothelial cells through several mechanisms and different mediators: endothelial activation/damage may be mediated by C1q.

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“…ЦВБ -достаточно редкое проявление НПСКВ, его частота, по разным данным, колеблется от 7 до 12% [54][55][56][57]. Клиническими проявлениями цереброваскулярной васкулопатии могут быть инсульт, очаговая ишемия, тромбоз венозного синуса.…”

Section: таблицаunclassified

Neuropsychic manifestations in the Kyrgyz cohort of patients with systemic lupus erythematosus

Koilubaeva

Решетняк

Асеева³

et al. 2019

Naučno-praktičeskaâ revmatologiâ

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The aimof the investigation was to study neuropsychic manifestations (NPM) of systemic lupus erythematosus (SLE) in the Kyrgyz cohort of patients.Material and methods.The prospective study included 460 patients with a reliable diagnosis of SLE, verified according to the diagnostic criteria ACR (1997) and SLICC (2012), observed in the clinic of the Academician M. Mirrakhimov National Center of Cardiology and Therapy from january 2012 to december 2017. Signs of nervous system damage were revealed in 103 (22.39%) of 460 patients with SLE. Classification criteria of ACR (1999) were used to assess neuropsychic manifestations of SLE, which were diagnosed by the psychiatrist according to ICD-10. Cognitive disorders were detected by a psychologist using a specific test Mini-Mental State Examination (MMSE; mini-scheme study of the mental state of the patient).Results and discussion.Various signs of NPM SLE were revealed in 103 (22.39%) of 460 patients with SLE. Groups of patients with and without NPM SLE at the beginning of the study were comparable in age, time from the appearance of the first signs of SLE to the verification of the diagnosis and the value of the SLEDAI-2K index (p>0.05). Acute variant of SLE course was significantly associated with NPM SLE. The acute course of SLE was observed in 56 (54.38%) of the 103 patients with NPM SLE and 109 (30.53%) of the 357 patients without NPM SLE [odds ratio (OR) 2.71, 95% confidence interval (CI) of 1.73–4.24; p<0.001]. Subacute and chronic course of the disease was diagnosed in a similar number of patients with NPM SLE – 24 (23.30%) and without NSAIDS – 23 (23.33%) (OR 5.75 95% CI 3.54–9.34; p<0.001). In the majority of patients the central nervous system (CNS) lesions prevailed. It was present in 71 (68.93%) patients, peripheral nervous system (PNS) pathology was less frequent and revealed in 32 (31.07%) of 103 cases, and 4 (3.88%) patients had combined CNS and PNS lesions. One manifestation of NPM SLE was detected in 37 (52.11%) patients, two – in 15 (21.13%), three – in 14 (19.72%) and four – in 5 (7.04%) of 71 patients with CNS lesions. According to the criteria of ACR (1999) in 103 patients 155 different NPM SLE were diagnosed: CNS disorders – in 123 (79.35%) and PNS pathology – in 32 (20.65%). The frequency of focal and diffuse CNS disorders was 61.79% and 38.21%, respectively. Cerebrovascular disease (CVD) was diagnosed most frequently – in 33 (43.42%) of the 76 cases of focal neuropsychiatric CNS disorders. Clinical manifestations of CVD were mainly characterized by discirculatory encephalopathy – in 30 (90.91%), less often – by ischemic stroke in the middle cerebral artery basin on the left – in 2 (6.06%) and transient ischemic attack – in 1 (3.03%) of 33 CVD cases. One manifestation was present in 52.11% of patients with CNS pathology. In other cases, there were more symptoms of its damage. Among the 47 diffuse lesions, neuropsychic disorders of the psychosis type prevailed, the main manifestations of which were visual and auditory hallucinations – in 34 (72.34%) patients.Conclusion.NPM SLE was identified in 22.39% of patients. Acute variant of SLE and very high activity was associated with NPM SLE. The risk of NPM SLE developing in very high activity of SLE was increased by 5.75 times. In the vast majority of cases in Kyrgyz patients there was CNS involvement (68.93%), twice less – PNS damage (31.07%), combined lesion of PNS and CNS was noted less frequently (3.88%). 47.89% of patients had more than one manifestation of NPM SLE. In most patients with diffuse NPM SLE, psychosis was observed in the form of visual and auditory hallucinations (72.34%), and focal ones were presented by CVD (43.42%), usually in the form of discirculatory encephalopathy (90.91%).

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Aggressive CNS lupus vasculitis in the absence of systemic disease activity

Cited by 14 publications

References 8 publications

Neuropsychiatric Lupus: A Challenging Journey of a Patient With Pulmonary Tuberculosis

Neuropsychiatric Lupus: A Challenging Journey of a Patient With Pulmonary Tuberculosis

Fulminant neurologic manifestation of Systemic Lupus Erythematosus

Neuropsychic manifestations in the Kyrgyz cohort of patients with systemic lupus erythematosus

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