Central nervous system symptoms in a population of Behcet's disease patients with refractory uveitis treated with cyclosporine A

2001 ◽  
Vol 29 (5) ◽  
pp. 335-336 ◽  
Clinics ◽  
2007 ◽  
Vol 62 (6) ◽  
pp. 685-690 ◽  
Author(s):  
Romy Christmann Souza ◽  
Laís Lage ◽  
Cláudia Goldesntein-Schainberg ◽  
André Regis Macedo ◽  
Solange Carrasco ◽  
...  

1993 ◽  
Vol 33 (4) ◽  
pp. 287-293 ◽  
Author(s):  
S.P. Morrissey ◽  
D.H. Miller ◽  
R. Hermaszewski ◽  
P. Rudge ◽  
D.G. MacManus ◽  
...  

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 848.2-848
Author(s):  
N. Plaza Aulestia ◽  
M. J. Pérez ◽  
S. Rodríguez Montero ◽  
J. L. Marenco

Background:Behçet’s disease (BD) is a variable vessel vasculitis characterized by mucocutaneous lesions (oral and genital ulcers). In addition, it can affect other systems such as vascular, ophthalmological, gastrointestinal tract or central nervous system (CNS). The latter being one of the most serious manifestations of Behçet’s disease. We present a descriptive observational study of a cohort of patients diagnosed with Behçet’s disease with CNS involvement.Objectives:Our objective is to describe the prevalence and characteristics of various clinical manifestations in patients with Behçet.Methods:Observational, descriptive, cross-sectional, retrospective design performed in patients with Behçet’s disease in follow-up by the Rheumatology department of the Hospital de Valme, until October 2019. The following information is collected from medical records; sex, mean age at diagnosis, clinical characteristics, HLA B51 positivity and treatment.Results:Thirty-three patients were diagnosed with Behçet’s disease, with a mean age at diagnosis of 36.22 +/- 10.94 years, female predominance 19 (57.57%). Regarding the determination of HLA B 51; 5 (15.15%) positive, 6 (18.18%) negative and 22 (66.66%) not determined. The following information about clinical manifestations is collected:Clinical manifestations n (%)Oral aphtosis33 (100)Genital ulceration23 (69.69)Skin (Pseudofolliculitis/Erythema Nodosum)12 (36.36)Ocular22 (66.66)Anterior Uveitis8/22 (36.36)Posterior uveitis3/22 (13.63)Panuveitis5/22 (22.72)Vitritis1/22 (4.54)Retinal vasculitis6/22 (27.27)Vascular7 (21.21)Arthralgias/arthritis20 (60.60)Gastrointestinal2 (6.06)Central Nervous System9 (27.27)Demyelinating lesions5/9 (55.55)Stroke2/9 (22.22)Epilepsy1/9 (11.11)Vasculitis1/9 (11.11)We identified 9 patients with CNS involvement; 5 demyelinating lesions (55%), 2 strokes (22%), 1 epilepsy/seizure (11%) and 1 case of CNS vasculitis (11%). Fifty-five percent of the patients were female (55%). Two patients were positive for HLA B51 (22%) and the rest had not been determined. Regarding treatment, all patients received both, oral and intravenous, corticosteroids (prednisone, methylprednisolone) and seven of them received DMARDs; 2 methotrexate (28%), 2 cyclosporine (28%), 1 sulfasalazine (14%), 1 azathioprine (14%) and 1 mercaptopurine (14%, due to intestinal involvement). Two of them needed biologic DMARD with TNF inhibitor agents (Infliximab and Adalimumab).Conclusion:Although neurological involvement is not common in BD, we describe higher prevalence (22%) than other series in the literature1,2. Given the heterogeneity of the clinical presentation there is a wide differential clinical diagnosis to rule out; demyelinating diseases (multiple sclerosis), such as demyelinating lesions presented in 5 of our patients (55%), infectious diseases (tuberculosis), other autoimmune diseases (Vogt–Koyanagi–Harada Syndrome, Eales Sd) or ischemic stroke due to atherosclerosis3.References:[1] 10.1016/B978-0-7020-4088-7.00110-3[2]10.1046/j.1365-4362.2003.01741[3]10.1007/s00415-013-7209-3Disclosure of Interests:NAHIA PLAZA AULESTIA: None declared, MARÍA JOSÉ PÉREZ: None declared, Sergio Rodríguez Montero: None declared, Jose Luis Marenco Speakers bureau: ABbvie, Pfzer, lilly


2019 ◽  
Author(s):  
Mariem Kchaou ◽  
Khadija Bahrini ◽  
Meriam Belghith ◽  
Aroua Cherif ◽  
Olfa Maghrebi ◽  
...  

Abstract Background: When the central nervous system (CNS) is the primary affected site in an initial attack of Behcet’s disease (BD), the differential diagnosis is particularly challenging. Some cases remain unclassified or qualified as probable Neuro-Behçet disease (NBD). Moreover, it was demonstrated that cytokines play a crucial role in the pathogenesis of NBD. We therefore studied peripheral and cerebrospinal inflammatory profile of these patients. Methods: Twenty two parenchymal NBD patients diagnosed according to the international consensus recommendation criteria and classified into definite (d-NBD; n= 13) and probable (p-NBD ; n=9) were sampled at their first neurological symptoms and compared with healthy control subjects (n=10). Oligoclonal bands of IgG were detected by isoelectric focusing on agarose and immunoblotting of matched serum and Cerebrospinal fluid (CSF) sample pairs. Cytokines and transcription factors related to TH1, TH2, TH17 and T regulatory populations were studied by quantitative RT-PCR in the CSF. Results: Oligoclonal bands (OCB) were present in only 1/22 patients. Two d-NBD patients had OCB in the CSF showing pattern 4. In NBD CSF samples, INF gamma, IL-17 and IL-10 expressions were significantly elevated compared with controls, however no difference in those cytokine expressions was observed between d-NBD compared to p-NBD. The most stricking finding was the significant increase of CSF IL-6 in d-NBD compared to p-NBD. Conclusion:These results indicate the rare presence of OCB in parenchymal NBD patients. Additionally, CSF IL-6 could help us to identify definite NBD. Keywords: Behçet’s disease, central nervous system, cerebrospinal fluid, cytokine.


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