Abstract
Behçet's disease (BD) is a multisystemic vasculitis disorder of almost unknown etiology, which involves small and large vessels and affects both veins and arteries. BD is characterized by recurrent oral aphthae (the main and most recurrent symptoms), genital ulcers, variable skin lesions, arthritis, uveitis, and thrombophlebitis. Other reported symptoms concern the involvement of the gastrointestinal and the central nervous system. Neuro-Behçet's disease (NBD) is one of the main causes of long-term morbidity and mortality, making its prompt recognition and treatment fundamental to attaining a better outcome. As pointed out by Kalra et al., there are definite consensus statements for BD, but less data are available for NBD. A multidisciplinary team of rheumatologists, dermatologists, ophthalmologists, neurologists, cardiovascular surgeons, and gastroenterologists, often led by rheumatologists, participate in the management of patients with BD and NBD.
Highlights
Neuro‐Behçet’s disease (NBD) is one of the main causes of long‐term morbidity and mortality, making its prompt recognition and treatment fundamental to attaining a better outcome
This review aims to define NBD and its clinical and pharmacological aspects
A genetic contribution has been found; human leucocytic antigen (HLA)-B5 allele, and, HLA-B51, was found to be the most strong genetic factor related to the onset of BD.[19]
Summary
7% to 49%; superficial phlebitis, deep vein thrombosis, large vein thrombosis, arterial thrombosis, and aneurysms are common and might be included in the diagnostic criteria of BS.[38,39] Cardiac involvement includes pericarditis, myocarditis, endocarditis, mitral valve prolapse, valve lesions, intracardiac thrombosis, endomyocardial fibrosis, myocardiopathy, and coronary artery lesions.[40]. Gastrointestinal symptoms have been described; the whole gastrointestinal tract might be involved but usually ulcers are most common in the terminal ileum. The main symptoms include abdominal pain, vomiting, diarrhea, bloating, or bleeding.[41]
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