Diagnosis/Classification Criteria for Behcet's Disease

Historical Background. The ISG criteria for Behcet's, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet's Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For ICBD, vascular lesions were added, while oral aphthosis is no more mandatory. Getting 3 or more points diagnose/classify the patient as BD (genital aphthosis 2 points, eye lesions 2 points, and the remaining each one point). Performance and Comparison of ISG and ICBD. Their sensitivity, specificity, and accuracy (percent agreement), were tested in three independent cohort of patients from Far-East (China), Middle-East (Iran), and Europe (Germany). The sensitivity for ISG was respectively 65.4%, 78.1%, 83.7% and for ICBD 87%, 98.2%, and 96.5%. The specificity for ISG was 99.2%, 98.8%, 89.5% and for ICBD 94.1%, 95.6%, and 73.7%. The accuracy for ISG was 74.2%, 85.5%, 85.5% and for ICBD 88.9%, 97.3%, and 89.5%. Conclusion. ICBD has better sensitivity, and accuracy than ISG.

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Behçet's Disease from Rheumatology and Neurology Perspectives with Special Reference to Children

Journal of Pediatric Neurology ◽

10.1055/s-0039-1692451 ◽

2019 ◽

Vol 18 (04) ◽

pp. 175-181

Author(s):

Yaşar B. Turgut ◽

Ayşe F. Tosun ◽

Beste K. Yüzbaşı ◽

Hasan Tunca

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

International Study ◽

Skin Lesions ◽

Disease Classification ◽

Behcet's Disease ◽

Male Predominance ◽

Pediatric Age Group ◽

Pathergy Test

AbstractBehçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.

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Behcet’s disease: a review

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20214828 ◽

2021 ◽

Author(s):

Daniela Ann Reyes-Weaver ◽

Kevin Luis Plata-Jimenez ◽

Raul Melo-Acevedo

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Age Of Onset ◽

Behçet's Disease ◽

Systemic Vasculitis ◽

Skin Lesions ◽

Vascular Lesions ◽

Severe Condition ◽

Behcet's Disease ◽

Oral Ulcers

Behcet's disease (BD) is a complex systemic vasculitis with an etiopathogenesis that remains unclear. It has a strong geographic association as well as a genetic propensity linked to the HLA-B51 factor and interactions between genetic and environmental factors. The typical age of onset is 25 to 40 years old and it is more common in men, who also have a more severe condition. Occlusive vasculitis is the hallmark of this condition, which can affect vessels of all diameters. Oral ulcers, genital ulcers, skin lesions, pathergy reaction as well as involvement of other systems and organs such as ophthalmic, neurological and vascular lesions, among others, are used to make the diagnosis. Uncontrolled neutrophil activation, activation of the humoral and cell immune systems, toxic proteins and infectious agents such as herpes simplex and streptococci are all involved in their pathophysiology. Due to the heterogeneity and several systems affected, the treatment is individualized and focused on treating each clinical manifestation.

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Behçet’s Disease as a Model of Venous Thrombosis

The Open Cardiovascular Medicine Journal ◽

10.2174/1874192401004010071 ◽

2010 ◽

Vol 4 (1) ◽

pp. 71-77 ◽

Cited By ~ 6

Author(s):

Micaela La Regina ◽

Armen Yuri Gasparyan ◽

Francesco Orlandini ◽

Domenico Prisco

Keyword(s):

Venous Thrombosis ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Skin Lesions ◽

Chronic Inflammatory Disease ◽

Vascular Lesions ◽

Behcet's Disease ◽

Therapeutic Measures ◽

Arterial Aneurysms

Behçet’s disease (BD) is a chronic inflammatory disease of unknown aetiology characterized by recurrent oral, genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in BD. The frequency of vascular lesions in BD, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. In this review, various factors of thrombogenesis in BD, particularly pro- and antithrombotic endothelial and non-endothelial factors, factors of coagulation, platelet activation and rheological changes are presented and discussed from positions of Virchow’s triad of venous thrombosis. Despite advances in understanding of thrombogenesis in BD, still many issues of diagnosis and targeted preventive and therapeutic measures remain unresolved. Further studies are needed to clarify the pathobiology of BD-related thrombosis and to provide the clinicians with recommendations over the utility, safety and effectiveness of the antithrombotic therapy in BD.

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Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report

Anais Brasileiros de Dermatologia ◽

10.1590/abd1806-4841.20131930 ◽

2013 ◽

Vol 88 (3) ◽

pp. 448-451 ◽

Cited By ~ 4

Author(s):

Maraya de Jesus Semblano Bittencourt ◽

Carolina Moraes Dias ◽

Thaiane Lima Lage ◽

Renata Silva Barros ◽

Otavio Augusto Gomes Paz ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Vena Cava ◽

Skin Lesions ◽

Vascular Lesions ◽

Hepatic Veins ◽

Budd Chiari Syndrome ◽

Behcet's Disease ◽

Chiari Syndrome

Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in Behçet's disease. The frequency of vascular lesions in Behçet's disease, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. Budd-Chiari syndrome is a rare and serious complication of Behçet's disease and implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. We report a case of a 25-year-old man with Behçet's disease that developed Budd-Chiari syndrome. The correlation of dermatological, pathological and imaging studies confirmed the diagnosis.

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A rare coexistence of Behcet’s disease and Graves’ thyrotoxicosis in a young man: a case report

Oxford Medical Case Reports ◽

10.1093/omcr/omz132 ◽

2020 ◽

Vol 2020 (1) ◽

Author(s):

Hareesh Joshi ◽

Imran Shahriar ◽

Poonam Sharma ◽

Satyanarayana V Sagi ◽

Samson O Oyibo

Keyword(s):

Behçet’S Disease ◽

Inflammatory Markers ◽

Behcet’S Disease ◽

Clinical Symptoms ◽

Behçet's Disease ◽

Autoimmune Disorder ◽

Skin Lesions ◽

Behcet's Disease ◽

Pathergy Test ◽

Vasculitic Disorder

Abstract Behcet’s disease is a recurrent systemic vasculitic disorder. It manifests most commonly in the form of skin lesions, oral and genital ulcers and uveitis. Graves’ thyrotoxicosis is an autoimmune disorder characterized by excessive production of thyroid hormones. We present a case of a 41-year-old male of Turkish descent who had symptoms of arthralgia, rash, palpitations and weight loss. Bloods tests showed raised inflammatory markers and biochemical evidence of severe autoimmune thyrotoxicosis. The patient was HLA-B51-negative, and pathergy test was inconclusive. A diagnosis of Behcet’s disease was made on constellation of clinical symptoms. The patient was treated with carbimazole and prednisolone followed by azathioprine. The coexistence of Behcet’s disease and Graves’ disease in the same patient is very rare. Further studies are required to determine if there is a pathological association between these two conditions.

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Behçet’s disease in clinical practice

Kazan medical journal ◽

10.17816/kmj1968 ◽

2013 ◽

Vol 94 (4) ◽

pp. 545-548

Author(s):

R F Khamitov ◽

L Y Palmova ◽

Z N Yakupova ◽

E R Khasanova

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Retinal Vasculitis ◽

Clinical Manifestations ◽

Specific Treatment ◽

Skin Lesions ◽

Female Ratio ◽

Behcet's Disease ◽

Pathergy Test

Behçet’s disease is a chronic relapsing multisystemic vasculitis involving vessels of different diameter. The disease is characterized by involvement of skin and mucosa, eyes, gastrointestinal tract, joints, vessels, genitourinary system and neurological symptoms. According to data, male/female ratio is 2-10:1 The etiology of the disease is still unclear. Diagnosis is mainly based on thorough analysis of clinical manifestations. According to the International Study Group for Behçet’s Disease Diagnostic Criteria, combination of recurrent oral aphthosis and at least any two of the following: severe or scarring genital aphthosis, eye lesions (posterior uveitis, retinal vasculitis), skin lesions (nodular erythema, pseudofolliculitis, acne-like lesions) and positive pathergy test is needed to classify the patient as a patient with Behçet’s disease. Several clinical subtypes of Behçet’s disease are distinguished depending on predominant involvement of different organs and systems, including skin and mucosa, joints, eyes, or neurological manifestations. There are no specific laboratory tests for Behçet’s disease. The treatment approaches are variable depending on clinical manifestations. The effect of systemic corticosteroids, colchicine, azathioprine, chlorambucil, cyclophosphamide, sulfasalazine, methotrexate, cyclosporine, infliximab was proven. All the abovementioned requires the good clinical knowledge on Behçet’s disease from practicing doctors of different specialties, particularly - physicians. A case of highly active acute Behçet’s disease with nose, pharynx, eye and oral cavity mucosa, scrotum skin involvement is presented in the article. Questions of differential diagnosis are reviewed, specific treatment program with efficacy analysis is presented.

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Infliximab in the treatment of Budd-Chiari syndrome in Behçet’s disease

Reumatismo ◽

10.4081/reumatismo.2019.1153 ◽

2019 ◽

Vol 71 (3) ◽

pp. 163-165 ◽

Cited By ~ 2

Author(s):

L.B. Santos ◽

J. Rigueira ◽

A.P. Vilas

Keyword(s):

Behçet’S Disease ◽

Clinical Remission ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Skin Lesions ◽

Vascular Lesions ◽

Budd Chiari Syndrome ◽

Necrosis Factor Alpha ◽

Behcet's Disease ◽

Chiari Syndrome

Behçet’s disease is a chronic autoinflammatory disorder that usually presents with recurrent oral and genital ulcers, uveitis, skin lesions and arthritis. Any organ, however, can be involved and in a subgroup of patients the large vascular lesions are the predominant feature (vasculo-Behçet disease). We report a case of a 27-yearold man with Behçet’s disease who developed Budd-Chiari syndrome while on immunosuppressive therapy. Infliximab resulted in a rapid and total clinical remission. Literature data have shown that antitumor necrosis factor alpha (anti-TNF-α) agents including infliximab can suppress disease activity but there is no long-term experience on tapering and/or discontinuation of treatment under clinical remission. Our case supports the use of infliximab in vasculo-Behçet patients.

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Prevalance of pathergy test positivity in Behcet's disease in Turkey

Journal of the European Academy of Dermatology and Venereology ◽

10.1046/j.1468-3083.2003.00577_2.x ◽

2003 ◽

Vol 17 (2) ◽

pp. 228-229 ◽

Cited By ~ 7

Author(s):

B Dogan ◽

O Taskapan ◽

Y Harmanyeri

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Behcet's Disease ◽

Pathergy Test

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Immunopathogenesis of Ocular Behçet’s Disease

Journal of Immunology Research ◽

10.1155/2014/653539 ◽

2014 ◽

Vol 2014 ◽

pp. 1-13 ◽

Cited By ~ 11

Author(s):

Un Chul Park ◽

Tae Wan Kim ◽

Hyeong Gon Yu

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Retinal Vasculitis ◽

Skin Lesions ◽

Unknown Etiology ◽

Ocular Involvement ◽

Inflammatory Disorder ◽

General Hypothesis ◽

Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

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