Behçet's syndrome

doi:10.1016/j.berh.2008.08.005

Best Practice & Research Clinical Rheumatology

Volume 22, Issue 5, October 2008, Pages 793-809

https://doi.org/10.1016/j.berh.2008.08.005 Get rights and content

Recent epidemiological work suggests that genetic background overrides environmental factors in the pathogenesis of Behçet's syndrome (BS). There are at least two clusters of disease expression. The first is the cluster of superficial vein thrombosis, deep vein thrombosis and dural sinus thrombi; the second cluster is that of acne, arthritis and enthesitis. The association of antibodies to anti-Saccharomyces cerevisiae antibodies and the presence of inflammatory bowel disease is perhaps another such cluster. The presence of such clusters suggests that there might be more than one disease mechanism operative in this complex disorder. There is a recent trend to classify BS with the autoinflammatory disorders. However, practically all autoinflammatory conditions are recurrent fever syndromes of children, and are genetically linked to well-defined loci; none of this is true for BS. Recent guidelines from the European League Against Rheumatism are quite useful for the management of the disease in organ systems other than the vascular, neurological and gastrointestinal systems, because of the lack of controlled studies related to these latter pathologies.

Introduction

In 1937, Hulusi Behçet described three patients with a triple symptom complex of aphthae, genital ulcers, and hypopyon uveitis.¹ Subsequent studies showed this entity to be a multisystem disease characterized by variable clinical manifestations. Almost all patients have recurrent oral aphthae, followed in frequency by genital ulcers, variable skin lesions, arthritis, uveitis, thromboplebitis, and gastrointestinal and central nervous system involvement. Behçet's syndrome (BS) is a systemic vasculitis of small and large vessels, of both the venous and arterial side, with an unknown aetiology.2, 3

This review of BS focuses on recent developments in the current understanding of the epidemiology, describing the major vessel involvement and the diagnostic criteria, and addressing the evidence-based therapies, as well as new approaches such as biological agents in the treatment.

Section snippets

Epidemiology

BS is most commonly seen in those countries that extend from the Mediterranean to the Far East along the ancient trading route known as the ‘silk road’. This suggests that the putative agent(s), including several genetic factors such as HLA-B51, spread along this route.⁴

The prevalence of BS was between 20 and 421 per 100,000 adults in five field surveys carried out in Turkey (Table 1)5, 6, 7, 8, 9; there was not a single patient among 47,000 children in another field survey from the same

Clinical manifestations

Mucocutaneous features are the most common presenting symptoms of the disease; eye, vascular and neurological involvement are the most serious.

Pathology and pathogenesis

Although vascular injury is quite common and can involve all sizes of vessels, both on the venous and the arterial side, frank necrotizing vasculitis of the small and medium-sized arteries of the type seen in anticardiolipin and antineutrophilic antibodies (ANCA)-associated vasculitides is infrequent. Similarly infrequent are giant cells and immune-complex-type cutaneous venulitis. These make the vascular involvement in BS rather unique.⁵⁴ Furthermore, there is little evidence for vasculitis in

Laboratory investigations

There are no specific laboratory findings. A moderate anaemia of chronic disease and leucocytosis is found in around 15% of patients. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are usually moderately elevated but do not correlate well with disease activity.⁷² Serum immunoglobulins are sometimes elevated; complement levels might also be high. Autoantibodies such as rheumatoid factor (RF), antinuclear antibody (ANA), anticardiolipin and antineutrophilic antibodies

Diagnosis and differential diagnosis

As there are no specific symptoms and signs, the diagnosis of BS is based on the recognition of a group of clinical features. In 1990, the International Study Group published a set of diagnostic (classification) criteria based on data from a computer analysis of clinical features in 914 patients with BS and 308 diseased controls with features that can be confused with Behçet's syndrome from seven countries around the world.⁷³ As these new criteria, which had a sensitivity of 91% and specificity

Prognosis and management

With time, BS goes into a complete remission in many patients.⁸¹ Younger patients and men generally have more severe disease, demonstrating an increased frequency both of mortality and of morbidity related to eye, vascular and neurological disease than women and older patients. Currently, loss of useful vision is seen in less than 10–15% of patients with eye involvement, compared with 75% of such patients 20–30 years ago. Whereas many patients with BS, especially older females, can be managed

Established (conventional) therapies

Recently, the European League Against Rheumatism (EULAR) has published guidelines for managing BS.⁹⁸ These provide good guidance for the management of BS in organ systems other than the vascular, neurological and gastrointestinal systems (there is a lack of controlled studies relating to these pathologies). Topical measures, including local steroid application, are sufficient for isolated oral and genital ulcers.

As the main pathology is inflammation of the vessel wall and lack of

Conflict of interest

None.

Role of the funding source

None.

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Cited by (148)

Orbital myositis as an initial manifestation of Behcet's disease
2022, American Journal of Ophthalmology Case Reports
Citation Excerpt :
The systemic manifestations of Behcet's disease (BD) include recurrent oral aphthae, followed in frequency by genital ulcers, variable skin lesions, arthritis, uveitis, thrombophlebitis, and gastrointestinal and central nervous system disorders.1
We report the case of a 32-year-old woman with orbital myositis prior to onset of Behcet's disease (BD).
A 32-year-old woman was referred to our hospital for a complaint of right eyelid swelling, eye pain, and diplopia. Her best-corrected visual acuity was 20/32 on the right, and 20/16 on the left. She was diagnosed as idiopathic orbital inflammation, and received two pulses of intravenous administration of methylprednisolone, followed by oral prednisolone. Three months later, she developed bilateral orbital myositis, and received one more pulse therapy, followed by oral prednisolone and cyclosporin. About one year after the first visit, oral aphthae, genital ulcers, and folliculitis-like skin rash appeared, and the physician diagnosed incomplete type BD. The patient had no uveitis during the disease course. Orbital inflammation and systemic manifestations of BD were ultimately well controlled with small doses of prednisolone.
BD may develop during the course of orbital inflammation.
Predictive Factors of Severe Behçet's disease: A Longitudinal, Prospective Cohort Followed Between 1981–2020
2022, Reumatologia Clinica
Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described.
To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort.
A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score >4 points.
We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N = 243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR = 2.004, p = 0.024), increasing with age (OR = 1.021 per year, p = 0.037), in the presence of skin manifestations (OR = 4.711, p < 0.001), specifically erythema nodosum (OR = 8.381, p < 0.001), and pseudofolliculitis (OR = 2.910, p < 0.001).
In the multivariate model, variables independently associated with severe BD were male gender (Adjusted OR = 1.961, p = 0.047), erythema nodosum (Adjusted OR = 8.561, p < 0.001) and pseudofolliculitis (Adjusted OR = 2.372, p = 0.007).
Male gender, erythema nodosum, and pseudofolliculitis were independently associated with severe BD forms and therefore should serve as warning signs to the clinician.
La enfermedad de Behçet (EB) es una vasculitis sistémica de causa desconocida. El espectro de la enfermedad abarca desde manifestaciones mucocutáneas hasta otras enfermedades de órganos con morbilidad relevante. Se han descrito asociaciones entre la gravedad de la enfermedad y el sexo masculino, la edad de inicio más temprana y la presencia de eritema nudoso.
Evaluar los factores clínicos asociados con las manifestaciones de enfermedad grave en una cohorte de un solo centro.
Estudio de cohorte longitudinal, prospectivo y unicéntrico con pacientes seguidos en una clínica ambulatoria especializada entre 1981 y 2020. La EB grave se definió como una puntuación Krause total clinical severity score ≥ 4 puntos.
Se incluyeron 243 pacientes, de los cuales el 31% eran varones, con un tiempo de seguimiento medio de 14,6 años. En cuanto a las manifestaciones orgánicas, todos los pacientes presentaron manifestaciones mucosas (n = 243, 100%), 133 (55%) piel, 104 (43%) articular, 71 (29%) ocular, 48 (20%) afectación vascular, 47 (19%) neurológica, 22 (9%) gastrointestinal y 1 (0,4%) cardiaca por EB; 156 (64%) pacientes fueron clasificados como con EB grave. La EB severa fue más frecuente en hombres (OR = 2,004, p = 0,024), aumentando con la edad (OR = 1,021 por año, p = 0,037), en presencia de manifestaciones cutáneas (OR = 4,711, p < 0,001), específicamente eritema nodosum (OR = 8,381, p < 0,001) y pseudofoliculitis (OR = 2,910, p < 0,001).
En el modelo multivariado, las variables asociadas de forma independiente con el EB grave fueron el sexo masculino (OR ajustado = 1,961, p = 0,047), eritema nudoso (OR ajustado = 8,561, p < 0,001) y pseudofoliculitis (OR ajustado = 2,372, p = 0,007).
El sexo masculino, el eritema nudoso y la pseudofoliculitis se asociaron de forma independiente con formas graves de DB y, por lo tanto, deberían servir como signos de advertencia para el médico.
Behçet's disease phenotypes and clinical outcomes: A cohort study in egyptian patients
2021, Reumatologia Clinica
Behçet's disease (BD) is a systemic inflammatory disease with various presentations. The data on the course of BD in Egyptian patients are limited.
The objective of the study was to describe the evolution and association of the different phenotypes of BD.
This chronological cohort study included adult Egyptian patients suffering from BD. Demographic data and the chronological order of the disease's manifestations were collected.
The study included 233 patients. Their mean age at the onset of the disease was 26.3 ± 6.9 years. The mean duration from onset of the disease to meeting the criteria was 11.2 ± 30.3 months. The mean duration of the disease was 96.8 ± 72.2 months. On onset of the disease, the most common phenotypes were mucocutaneous (84.5%), musculoskeletal (15.9%), ocular (14.6%) and peripheral venous disease (PVD) (7.3%); on the other hand, pulmonary, peripheral arterial and great vessel phenotypes evolved several years after onset of the disease. The mean time from meeting the criteria to the evolution of a new phenotype was 53.8 ± 58.7 months. Associations between the different phenotypes were observed: PVD and superficial thrombophlebitis, peripheral arterial disease and PVD; another association was also observed between aortic involvement and cerebrovascular disease.
BD could continue to evolve several years after onset of the disease, making the previous belief about BD yield questionable. BD tends to respect the anatomy of the affected system. Some phenotypes tend to coexist, suggesting a shared aethiopathogeny and that the disease is of a systemic nature.
La enfermedad de Behçet (BD) es una enfermedad inflamatoria sistémica con diversas presentaciones. Los datos sobre el curso de la BD en pacientes egipcios son limitados.
El objetivo del estudio fue describir la evolución y la asociación de los diferentes fenotipos de BD.
Este estudio de cohorte cronológico incluyó pacientes egipcios adultos que sufren de BD. Se recopilaron datos demográficos y el orden cronológico de las manifestaciones de la enfermedad.
El estudio incluyó a 233 pacientes. Su edad media al inicio de la enfermedad fue de 26,3 ± 6,9 años. La duración media desde el inicio de la enfermedad hasta el cumplimiento de los criterios fue de 11,2 ± 30,3 meses. La duración media de la enfermedad fue de 96,8 ± 72,2 meses. Al inicio de la enfermedad, los fenotipos más comunes fueron los fenotipos mucocutáneos (84,5%), musculoesqueléticos (15,9%), oculares (14,6%) y la enfermedad venosa periférica (7,3%); por otro lado, la enfermedad pulmonar, arterial periférica y fenotipos de grandes vasos evolucionaron varios años después del inicio de la enfermedad. La duración media desde el cumplimiento de los criterios hasta la evolución de un nuevo fenotipo fue de 53,8 ± 58,7 meses. Se observaron asociaciones entre los diferentes fenotipos: enfermedad venosa periférica y tromboflebitis superficial, enfermedad arterial periférica y enfermedad venosa periférica; también se observó otra asociación entre la afectación aórtica y la enfermedad cerebrovascular.
La BD podría continuar evolucionando varios años después del inicio de la enfermedad, haciendo cuestionable la previa creencia del rendido BD. La BD tiende a respetar la anatomía del sistema afectado. Algunos fenotipos tienden a coexistir, lo que sugiere una etiopatogenia compartida y una naturaleza sistemática de la enfermedad.
Laboratory and clinical correlates of brain atrophy in Neuro-Behçet's disease
2020, Journal of the Neurological Sciences
Citation Excerpt :
Behçet's disease (BD) is a multi-systemic and recurrent auto-inflammatory disorder which affects people mainly originating from countries around the Silk Road, Japan, and North Africa. The prevalence of BD varies among regions, e.g., 20–141 per 100.000 in Turkey, 0.64 per 100,000 in the U.K., and 6.4 per 100,000 in Spain [1]. The classical triad of BD comprises oral, genital ulcers, and uveitis [1–3].
Diagnostic evaluation of patients with parenchymal Neuro-Behçet's disease (NBD) requires magnetic resonance imaging (MRI), neuro-ophthalmologic, and neuropsychological evaluation. In this study, we aimed to find out the ideal diagnostic method that most closely reflects the progress in cognitive disability and brain atrophy in NBD.
In this matched case-control study, we included patients with parenchymal NBD, Behçet's disease without neurological involvement (BD), rheumatoid arthritis, and healthy controls. Detailed ophthalmological examination, pattern-reversal visual evoked potentials (prVEP) test, optical coherence tomography (OCT), brain MRI volumetry and cognitive evaluation tests were performed. Disability status was assessed by revised EDSS.
Sixty-eight individuals (35 female, 33 male) were recruited. Mean ACE-R scores were significantly lower in the NBD group (NBD vs. healthy, 80±14.4, 93±4.9, p=0.002). prVEP values were similar across groups, but retinal nerve fiber layer thickness (RNFLT) were more frequently abnormal in the NBD group. We found considerable volume reduction in the brainstem, cerebellum, hippocampus, and thalamus in the NBD group. Regarding prVEP, 120 minutes P100 amplitude (p<0.001, r=0.97) and 60 minutes P100 amplitude values (p=0.006, r=0.90) were positively correlated with the total cerebral white matter volume.
Our results confirmed previous observations on cognitive dysfunction in patients with NBD. We reported MRI volumetry data of patients with parenchymal neuro-Behçet's disease for the first time and elucidated novel brain regions with atrophy. Clinically determined scores and OCT failed to predict the status of brain atrophy. prVEP P100 amplitude may be used as a surrogate marker of cerebral white matter involvement in NBD.
The link “Cancer and autoimmune diseases” in the light of microbiota: Evidence of a potential culprit
2020, Immunology Letters
Citation Excerpt :
BD is a rare vasculitis recently classified at the crossroad between autoimmune and autoinflammatory disorders [349] that may increase cancer risk. It is characterized by variable clinical manifestations, including oral and genital aphthae, cutaneous lesions, ocular, gastrointestinal, neurologic involvement, and arthritis [350]. Its prevalence in the Mediterranean, Central Asia, and in the Far East is significantly higher than in Europe and the United States [351].
Evidence establishes that chronic inflammation and autoimmunity are associated with cancer development and patients with a primary malignancy may develop autoimmune-like diseases. Despite immune dysregulation is a common feature of both cancer and autoimmune diseases, precise mechanisms underlying this susceptibility are not clarified and different hypotheses have been proposed, starting from genetic and environmental common features, to intrinsic properties of immune system. Moreover, as the development and use of immunomodulatory therapies for cancer and autoimmune diseases are increasing, the elucidation of this relationship must be investigated in order to offer the best and most secure therapeutic options.
The microbiota could represent a potential link between autoimmune diseases and cancer. The immunomodulation role of microbiota is widely recognized and under eubiosis, it orchestrates both the innate and adaptive response of immunity, in order to discriminate and modulate the immune response itself in the most appropriate way. Therefore, a dysbiotic status can alter the immune tonus rendering the host prone to exogenous or endogenous infections, breaking the tolerance against self-components and activating the immune responses in an excessive (i.e. chronic inflammation) or deficient way, favoring the onset of neoplastic and autoimmune diseases.
Behçet's disease patterns and subsets in a cohort of Egyptian patients
2019, Egyptian Rheumatologist
To study patterns and disease subsets of Behcet’s disease (BD) patients admitted to Cairo University Hospitals and to detect whether relapse of the disease will affect the same system every time or not.
A retrospective study involving 82 BD patients admitted to Cairo University Hospitals, from January 2000 to December 2014. They were reviewed to analyze the frequency of different disease manifestations and to find out disease patterns and subsets.
75 men and 7 women were included in the study, with a mean age of 34.2 ± 9.7 years. Their disease duration ranged from 1 to 34 years with a mean of 9.1 ± 6.9 years. Mucocutaneous manifestations were present in 82 patients (100%), ocular manifestations in 53 patients (64.4%), vascular manifestations in 49 patients (59.8%), and neurological manifestations in 9 patients (11%). Most of our patients, 48 (58.5%) had the same one system pattern throughout the disease course, 25 patients (30.5%) had two systems patterns and 9 patients (11%) had three systems patterns.
BD usually affects the same system throughout the disease course whether mucocutaneous, vascular, ocular or neurological and the most common pattern is the one system affection. This will help to predict the system that will be affected in each time the patient presents with a disease relapse. Also, it will help in differentiation between disease relapse and any associated other disease minimizing the need for and the cost of investigations. However, future studies on larger number of patients are recommended.

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2Behçet's syndrome

Introduction

Section snippets

Epidemiology

Clinical manifestations

Pathology and pathogenesis

Laboratory investigations

Diagnosis and differential diagnosis

Prognosis and management

Established (conventional) therapies

Conflict of interest

Role of the funding source

Seminars in Arthritis and Rheumatism

The Journal of Pediatrics

American Journal of Ophthalmology

American Journal of Medicine

Seminars in Arthritis and Rheumatism

The Journal of Urology

Human Pathology

The American Journal of Medicine

Lancet

Lancet

Uber rezidivierende, aphthose, dürch ein Virus verursachte Geshwure am Munde, am Auge und an den Genitalien

Dermatologische Wochenschrift

Behcet's disease

The New England Journal of Medicine

Behçet's syndrome

Behçet's disease, the Silk Road and HLA-B51: historical and geographical perspectives

Tissue Antigens

Silivri Fener koyu ve yoresinde Behçet hastaligi sikligi ve bu hastaligin toplum icinde taranabilmesinde kullanabilecek bir yontem

Cerrahpaşa Tip Fakültesi Dergisi

The prevalence of Behçet's syndrome in a rural area in northern Turkey

The Journal of Rheumatology

The prevalence of Behçet's disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey

Ophthalmic Epidemiology

Prevalence of Behçet's disease in Istanbul, Turkey

International Journal of Dermatology

Prevalence of Behçet's disease in rural western Turkey: a preliminary report

Clinical and Experimental Rheumatology

Prevalence of juvenile chronic arthritis and familial Mediterranean fever in Turkey: a field study

The Journal of Rheumatology

A nation wide survey of Behçet's disease in Japan

Japanese Journal of Ophthalmology

Prevalence of Behçet's syndrome in Hawai

Hawaii Medical Journal

Behçet's syndrome in 32 patients in Yorkshire

Annals of the Rheumatic Diseases

Epidemiologic and clinical aspects of Behçet's disease in a defined area of Northwestern Spain, 1988–1997

The Journal of Rheumatology

Epidemiological aspects of Behçet's disease in Galicia

The Journal of Rheumatology

Behçet's disease in southern Chinese patients

The Journal of Rheumatology

Prevalence of Behçet's disease among Iraqis

Advances In Experimental Medicine and Biology

Prevalence of Behçet's disease in an Arab community in Israel

Annals of the Rheumatic Diseases

Prevalence of Adamantiades-Behçet's disease in Germany and the municipality of Berlin: results of a nationwide survey

Clinical and Experimental Rheumatology

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: a seventeen-year population-based study

Arthritis and Rheumatism

Incidence and prevalence of Behçet's disease in a north American population

Arthritis and Rheumatism

Epidemiology of Behçet's disease in a multiethnic French population

Arthritis and Rheumatism

Behcet's disease in patients of West African and Afro-Caribbean origin

British Journal of Ophthalmology

Gastrointestinal involvement in Behçet's syndrome: a controlled study

Annals of the Rheumatic Diseases

A comparative study of the pathergy among Turkish and British patients with Behçet's disease

Annals of the Rheumatic Diseases

Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behçet's syndrome

Annals of the Rheumatic Diseases

The frequency of scarring after genital ulcers in Behçet's syndrome: a prospective study

International Journal of Dermatology

Histopathologic features of the spontaneous pustular lesions of Behçet's syndrome

International Journal of Dermatology

The usefulness of the nonspecific skin hyperreactivity (The pathergy test) in Behçet's disease in Turkey

Acta Dermatovener (Stockholm)

Uç nahiyede nuksi tavazzular yapan ve hususi bir virus tesiriyle umumi intan hasil ettigine kanaatimiz artan (Entite morbide) hakkinda

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Behçet's syndrome