Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis

Ishido, Mizuho; Horita, Nobuyuki; Takeuchi, Masaki; Shibuya, Etsuko; Yamane, Takahiro; Kawagoe, Tatsukata; Ishido, Takehito; Yoshimi, Ryusuke; Kirino, Yohei; Hirohata, Shunsei; Ishigatsubo, Yoshiaki; Takeno, Mitsuhiro; Kaneko, Takeshi; Mizuki, Nobuhisa

doi:10.1038/s41598-017-09938-z

Cited by 30 publications

(22 citation statements)

References 42 publications

(51 reference statements)

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“…NBD is subcategorized into parenchymal NBD (P-NBD) and nonparenchymal NBD (NP-NBD) types[21]. Furthermore, P-NBD is subdivided into acute P-NBD (A-P-NBD) and chronic progressive P-NBD (CP-P-NBD) [22,23]. A-P-NBD is characterized by acute meningoencephalitis with or without focal lesions.…”

Section: Discussionmentioning

confidence: 99%

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

et al. 2019

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Patient: Male, 67 Final Diagnosis: Unclassified vasculitis Symptoms: Fever • ocular pain • erythema • chest pain • headache Medication: — Clinical Procedure: Biopsy Specialty: Rheumatology Objective: Rare disease Background: Systemic vasculitides constitute heterogenous conditions affecting many organs and systems through blood vessel inflammation. Although there are some classifications for vasculitis, several vasculitides are “unclassified” because they cannot be clearly assigned to one of the known entities. Case Report: We report an autopsy case of a 67-year-old Japanese man who presented with fever, ocular pain, erythema, chest pain, and headache. The disease caused episcleritis, thrombophlebitis, extensive deep vein thrombosis, multiple pulmonary nodules and masses, hypertrophic pachymeningitis, and hyper-intensity areas in brain parenchyma on magnetic resonance images. Histopathology of the pulmonary nodule confirmed vasculitis affecting medium-to-small veins and arteries without necrotizing vasculitis or granulomatous inflammation. We diagnosed the patient with unclassified vasculitis based on the clinicopathological characteristics. Steroids in combination with immunosuppressants were used, but the disease was refractory and relapsing. The disease activity was eventually controlled with rituximab, but the patient died of bronchopneumonia. On autopsy, lung and brain findings indicated healed vascular inflammation. Conclusions: This is the first case report of unclassified vasculitis, which is characterized as medium-to-small-sized arteritis and phlebitis, causing episcleritis, thrombophlebitis, deep vein thrombosis, pulmonary vasculitis, and intracranial vasculitis. The clinical conditions share some similarities with granulomatosis with polyangiitis and Behçet’s disease; however, they meet no classification criteria of any specific vasculitis. More cases need to be analyzed to confirm our findings.

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Section: Discussionmentioning

confidence: 99%

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

et al. 2019

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“…The etiology is considered to involve vascular inflammation in the large/small veins [82]. CNS involvement, usually called neuro-Behçet disease (NBD), can present as an acute or chronic progressive type [83,84]. Acute NBD is characterized by acute meningoencephalitis with focal lesions on MRI and increased neutrophil cell counts in the CSF.…”

Section: Neuro-behçet Diseasementioning

confidence: 99%

Blood–brain barrier dysfunction in immuno-mediated neurological diseases

Shimizu

Nishihara

Kanda

2018

Immunological Medicine

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The blood-brain barrier (BBB) is the brain-specific endothelial cell barrier that is important for maintaining brain homeostasis and preventing the entry of toxic substances. Pathological BBB dysfunction is a critical step of the disease process in several immuno-mediated neurological diseases, including multiple sclerosis (MS), neuromyelitis optica (NMO), neuropsychiatric systemic lupus erythematosus (NPSLE) and neuro-Behçet diseases. The pathological findings from patients with secondary progressive (SP) MS, NMO and NPSLE showed leaky BBB in the active lesions. NMO is a disease with strong evidence of disease-specific and pathogenic autoantibodies (aquaporin 4 [AQP4] autoantibodies). In the development of NMO, circulating AQP4 autoantibodies need to pass through the BBB in order to reach AQP4 on the astrocyte endfeet. Strong evidence suggests that NPSLE is associated with the disruption of the BBB and NPSLE patients frequently have antibodies bound to endothelial cells in their sera. We recently identified two BBB-reactive autoantibodies in immuno-mediated neurological diseases: galectin-3 autoantibodies in SPMS and GRP78 autoantibodies in NMO. In the present review article, we describe the basic structure and cellular biology of the BBB, discuss recent insights regarding the pathophysiology of the BBB breakdown in the setting of immuno-mediated neurological diseases, and describe our recent findings of autoantibody-mediated BBB breakdown.

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“…First defined in 1954, [ 54 ], central nervous system (CNS) involvement (neuro-Behçet) occurs in 5–10% of patients, more frequently in males, and it usually occurs around 5 years after the onset of the disease [ 55 , 56 ].…”

Section: Audiovestibular Pathology Associated With Systemic Autoimmentioning

confidence: 99%

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

Girasoli

Cazzador

Padoan

et al. 2018

Journal of Immunology Research

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The prevalence of autoimmune diseases has been increasing over the last 20 years. The clinical presentation of this large and heterogeneous group of disorders depends on whether the involvement is organ-specific or non-organ-specific. Dizziness, vertigo, and disequilibrium are common symptoms reported by patients with vestibulocochlear involvement. The association of vertigo and autoimmune diseases has been largely documented, suggesting that autoimmune disorders could be overrepresented in patients with vertigo in comparison to the general population. The aim of this review is to present the recent literature findings in the field of autoimmune-mediated diseases with cochleovestibular involvement, focusing on the clinical presentation, diagnosis, and treatment of immune-mediated inner ear diseases including autoimmune inner ear disease (AIED), Meniere's disease, and bilateral vestibulopathy, as well as of systemic autoimmune diseases with audiovestibular disorders, namely, Behçet's disease, Cogan's syndrome, sarcoidosis, autoimmune thyroid disease, Vogt-Koyanagi-Harada syndrome, relapsing polychondritis, systemic lupus erythematosus, antiphospholipid syndrome, IgG4-related disease, and ANCA-associated vasculitides.

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Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis

Cited by 30 publications

References 42 publications

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

Blood–brain barrier dysfunction in immuno-mediated neurological diseases

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

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