Molecular Genetics (HLA) of Behçet’s Disease

Mizuki, Nobuhisa; Inoko, Hidetoshi; Ohno, Shigeaki

doi:10.1007/978-3-642-56455-0_14

Cited by 21 publications

(26 citation statements)

References 45 publications

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“…This triple‐symptom complex was first described by Hulusi Behçet 1 a Turkish dermatologist, as a separate disease entity. Later, other associated clinical features were described 2,3 …”

Section: Introductionmentioning

confidence: 99%

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Kaya

Türsen

Gürler³

et al. 2002

Clinical and Experimental Dermatology

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Genetic factors appear to be important in the pathogenesis of Behçet's disease. Although it is known to be strongly associated with HLA-B 51, the association of HLA class I antigens with specific clinical findings of the disease has not been studied extensively and the few studies are conflicting. The aim of this study was to investigate the association of HLA class I alleles with the manifestations of Behçet's disease in Turkish patients. Eighty-five patients with Behçet's disease were typed for HLA-A, B, and C antigens with the serologic, standard microlymphocytotoxicity technique. Possible associations of the HLA complex with clinical findings of Behçet's disease were examined. Statistically significant findings are as follows (P < 0.05): increased HLA-B 51 and decreased HLA-B35 frequency in patients with thrombophlebitis, increased HLA-A29 and decreased HLA-Bw6 frequency in patients with ocular involvement, decreased HLA-Cw2 frequency in patients with erythema nodosum, and decreased HLA-Cw 7 frequency in patients with genital ulceration. Of particular note, the results of this study suggest that the presence of HLA-B 51 and the absence of HLA-B35 can be regarded as laboratory risk factors of venous thrombosis in patients with Behçet's disease.

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“…This triple‐symptom complex was first described by Hulusi Behçet 1 a Turkish dermatologist, as a separate disease entity. Later, other associated clinical features were described 2,3 …”

Section: Introductionmentioning

confidence: 99%

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Kaya

Türsen

Gürler³

et al. 2002

Clinical and Experimental Dermatology

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“…3 This autoimmune disease may be familial and has a genetic basis. 4 It can also be considered multifactorial as genetic predisposition, abnormalities in both cellular and humoral immune system, and endothelial cell dysfunction are involved in its pathogenesis. 5 This unique disorder begins between the second and third decades of life (range 0-72 years) with a 3:2 male to female ratio.…”

mentioning

confidence: 99%

Neurotological status in Behçet’s disease and its ophthalmological correlates

Bayazıt¹,

Evereklioğlu

Özer

et al. 2004

Postgraduate Medical Journal

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Objective: To assess neurotological status and its ophthalmological correlates in Behçet's disease. Study design: A cross sectional study in which 17 patients with Behçet's disease and 13 healthy controls were included. Patients and methods: The patients were divided into two groups: those with and without end stage ocular involvement. Neurotological status was evaluated with audiological tests (pure tone and speech audiometry, tympanometry, short increment sensitivity index, tone decay, and evoked response audiometry), and Dix-Halpike positional testing. Results: The positional test was normal. The audiological test results of the patients with and without end stage ocular involvement were not significantly different (p.0.05). A sloping audiogram with bilateral symmetric and mild sensorineural hearing loss was the main audiogram obtained. There was no relationship between the presence or absence of the end stage ocular involvement and the otological parameters studied excluding I-III interval on evoked response audiometry (p.0.05). Conclusion: Although the main underlying pathogenetic factor in Behçet's disease is the autoimmune vasculitis, the mechanisms involved in the pathogenesis of neurological and ocular damage may be different. Alternatively, the differential involvement of certain organ systems may not reflect the nature of the disease process itself, but rather the manner in which each organ responds to injury.

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“…Recent studies suggest that MICA (major histocompatibility complex class I chain related gene A) which is located near the B51, may be the pathogenetic gene, as it occurs in a higher frequency than B51 in Japanese87 and Caucasoids,88 although this was not confirmed in Middle Eastern patients 89. Further studies from Japan suggest that the susceptibility locus for Behçet's disease may be located between the MICA and HLA-B genes 90…”

Section: Aetiology/pathogenesismentioning

confidence: 98%

Behçet’s disease

Kontogiannis

Powell

2000

Postgraduate Medical Journal

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Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies. (Postgrad Med J 2000;76:629-637)

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Molecular Genetics (HLA) of Behçet’s Disease

Cited by 21 publications

References 45 publications

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Neurotological status in Behçet’s disease and its ophthalmological correlates

Behçet’s disease

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