Manifestations neurologiques de la maladie de Behçet

Neurological manifestations of Behçet's disease (BD) occur in 5.3 to more than 50% of patients. They are divided into two major forms: “parenchymal” lesions, which include mainly meningoencephalitis as opposed to “extra-parenchymal” lesions (i.e. cerebral venous thrombosis and arterial aneurysms). Myelitis or peripheral neuropathy is exceptional. The neuro-Behçet syndrome (NBS) should be considered in the setting of neurological manifestations, particularly headache and pyramidal signs, in a young man diagnosed with BD. However, its recognition may be difficult when neurological manifestations are the presenting features of BD (one third of cases), and requires a thorough knowledge of clinical manifestations and morphological lesions. Thus, parenchymal NB lesions classically exhibit inflammatory characteristics on MRI and are located at the meso-diencephalic junction and in the brainstem, rarely with a supratentorial extension. Meningitis is not systematically associated, and may be absent in about 30% of cases. The pathogenesis of these lesions is incompletely understood, but inflammatory infiltrates include mainly neutrophils and activated T cells (mainly Th17). Differential diagnoses include infectious diseases (herpes, listeria, tuberculosis), and inflammatory diseases (i.e. multiple sclerosis and sarcoidosis). A prompt recognition of NBS should lead to initiate adequate therapies in order to limit the risk of sequelae, relapses or death.     

Manifestations oculaires de la maladie de Behçet

Eye involvement in Behçet's disease (BD) is frequent and an important cause of morbidity. The mean age at onset of uveitis is between 20 and 30 years in male and 30 years in female patients. Ocular involvement includes anterior, posterior or panuveitis. Uveitis may be the presenting manifestation of the disease in 20 % of cases or may appear 2 or 3 years after the disease onset. The estimated risk of blindness at 5 years ranges from 15 to 25 %. The main goals in the management of patients with BD uveitis are the rapid control of intraocular inflammation, the prevention of recurrent attacks, the achievement of complete remission, and preservation of vision. The medical treatment of patients with severe uveitis relies on the use of systemically administered drugs, including corticosteroids and cytotoxic agents. Anti-TNF agents and interferon-α seem to be efficient and well-tolerated alternative therapeutic options. Controlled clinical trials are mandatory to define the place of these new immunomodulatory agents in the therapeutic strategy, and especially their use as first-line therapy.     

Traitement de la maladie de Behçet

Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, vascular, neurological, articular, and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, non-steroidal anti-inflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapses, sight threatening eye disease, or irreversible organ damage. Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current non-specific immunosuppressive agents, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD.     

Thromboses artérielles multiples au cours de la maladie de Behçet

Behcet's disease (BD) is a multisystemic vasculitis. Its etiopathogeny remains unknown. Vascular involvement in BD is frequent and venous thrombosis is the most common manifestation (30 % of cases). Arterial involvement is rare (2.7 to 7 %). The latter is often severe and considered as a life threatening complication. Pathogenesis of thrombosis occurring in BD remains unclear. We report a 45-year-old man, from south of Tunisia, who presented a BD with a bifocal arterial involvement: right internal carotid thrombosis and bilateral proximal thrombosis of the two pulmonary arteries. Therapeutic strategies to address this multiple arterial involvement and the pathogenesis of thrombosis raise many questions.     

La maladie de Behçet chez la femme

Purpose

Behçet's disease (BD) is a multisystemic inflammatory disease characterized, on the one hand, by the frequency of benign mucocutaneous and articular manifestations, and on the other hand by the severity of ocular, neurological, vascular and digestive manifestations which may threaten functional or vital prognosis. The male BD predominance is obvious in many retrospective studies and a few studies only have focused on BD in women.

Methods

In this study, we report the epidemiological, clinical, outcome and therapeutic data of a series of 71 female BD compared to a series of 111 male BD. This retrospective study was conducted in the internal medicine department of Monastir university hospital over a 19-year period (1990 to 2008), including all patients followed-up and treated for BD.

Results

The comparison of clinical manifestations of BD between men and women showed the predominance of erythema nodosum (P = 0.016), necrotic pseudofolliculitis (P = 0.001), and joint involvement (P = 0.009) in women, while genital ulcers (P = 0.039), ocular involvement (P = 0.003) and vascular (P = 0.002) involvement were more common in men.

Conclusion

Our results confirmed the results of previous studies regarding the benign nature of BD in women compared to men.     

Atteintes cardiovasculaires de la maladie de Behçet

Vascular involvement is a common complication of Behçet's disease (BD) and affects up to 40% of BD patients. These complications worsen the prognosis of BD. The concept of vasculo-Behçet has been adopted for cases in which vascular complications dominate the clinical features. Vascular manifestations affect particularly young men, during the first years following onset of the disease. Venous complications are the most frequent vascular complications, affecting 14 to 40% of BD patients. Superficial and deep lower limb thrombosis is the most frequent venous complications but one third of venous thrombosis concern large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava, etc.). Budd-Chiari syndrome is the worst prognostic factor increasing mortality by 9 times. Arterial complications (2 to 17% of BD patients) include aneurysms and occlusions/stenosis. Main locations of arterial lesions are aortic (abdominal and thoracic), femoral, pulmonary and iliac arteries. Aneurysms are the most severe arterial complications, particularly pulmonary aneurysms associated with a high risk of massive bleeding. Cardiac complications (up to 6% of BD patients) include pericarditis, endocardial lesions (aortic regurgitation and less often mitral insufficiency), myocardial lesions (myocardial infarction, myocarditis and endomyocardial fibrosis) and intracardiac thrombosis (right ventricle and atrium). Coronary lesions complicated to myocardial infarction are the most severe cardiac complications. Treatment is based on corticosteroids and immunosuppressive drugs. The use of anticoagulation in venous thrombosis is still controversial.     

Les manifestations oculaires de la maladie de Behçet

Uveitis is the most common ocular symptom in Behçet's disease. It is considered as a diagnostic criterion of the disease. Anterior uveitis is always non granulomatous, and sometimes associated with hypopion. Posterior involvement may include vitritis, retinal infiltrates, sheathing of retinal veins, occlusive vasculitis, and macular edema. Behçet's disease is chronic, and characterized by a spontaneously relapsing and remitting course. Male patients with younger age at onset and worse visual acuity at presentation, have higher risk of visual loss over time. The main goals in the management of patients with Behçet's disease-associated uveitis are rapid suppression of intraocular inflammation, preservation of vision, prevention of recurrences, and achievement of remission sustained after discontinuation of treatment. New therapeutic strategies such as interferon alpha and TNF alpha blockers have dramatically improved the visual prognosis of patients with intraocular inflammation related to this chronic and potentially blinding condition.     

Épidémiologie de la maladie de Behçet

With more than 30 published prevalence estimates for Behçet's disease (BD), covering many different regions worldwide, the prevalence of BD is quite well described. Even though the interpretation of these data is complicated by between-study differences in methodology, which may substantially influence the results, these data suggest large geographic variations in frequency of BD, with prevalence rates of 20–420/100,000 inhabitants for Turkey, 2.1–19.5 for other Asian countries, 1.5–15.9 for southern Europe and 0.3–4.9 for northern Europe. Additional epidemiological studies or case series from North and South America, the Caribbean Islands, and individuals of sub-Saharan ancestry further suggest that the geographic distribution of BD is much wider than the boundaries of the ancient Silk Road. The few available incidence rates prevent from making strong inferences as to whether the frequency of BD has changed over time. Recent population-based studies of immigrants or migrant populations consistently indicate that migrants from areas of high BD prevalence remain at high risk for BD, which may even be close to the prevalence observed in their countries of origin. Genetic factors, which are not detailed in this review, seem to play a preponderant role in BD development, although they cannot explain the wide between-country disparities in BD prevalence. However, environmental risk factors, including infectious and non-infectious causes, remain poorly investigated and have not yet produced solid hints.     

Arthrite érosive du poignet : une manifestation rare de la maladie de Behçet

It is believed that arthritis in Behçet's disease is usually non erosive and not associated with crippling. We report a 38-year-old female who presented with Behçet's disease and an erosive arthritis of the left wrist.     

Maladie de Behçet de l’enfant

Behçet's disease is a chronic multisystem vasculitis of unknown etiology. The disease is commonly described in young adults but can occur in childhood. Diagnosis is based on clinical manifestations since there are no pathognomonic laboratory findings. Early diagnosis in children is challenging due to the insidious nature of the disease and the low sensitivity of adult criteria in the pediatric population. The purpose of this review is to describe the demographic and clinical features of Behçet's disease in childhood, its complications and recent advances in therapeutics highlighting differences with the adult onset disease.