Behcet disease

Jorn Oliver Sass Dr rer nat (Dr. Sass of Bonn-Rhein Sieg University of Applied Sciences has no relevant financial relationships to disclose.)
Francesc Graus MD PhD, editor. (Dr. Graus of the University of Barcelona has no relevant financial relationships to disclose.)
Originally released October 14, 1994; last updated August 13, 2018; expires August 13, 2021

This article includes discussion of Behcet disease, Behçet s syndrome, silk route disease, triple symptom complex, multiple symptom complex of Behcet, Morbus Behcet, oculobuccogenital syndrome, Adamantiades-Behcet disease, neurobehçet, neurobehcet, neuro-Behçet, neuro-Behcet, vasculo-Behcet, entero-Behcet, incomplete Behcet disease, complete Behcet disease, mucocutaneous form of Behcet disease, and neuro-ocular form of Behcet disease. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Behcet disease is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin that can affect the nervous system. Clinical and imaging evidence suggests that primary neurologic involvement may be subclassified into 2 major forms. The first form, which is seen in the majority of patients, may be characterized by a vascular-inflammatory central nervous system disease with focal or multifocal parenchymal involvement. The other form is caused by isolated cerebral venous sinus thrombosis and intracranial hypertension. In this article, the author describes the various clinical manifestations, summarizes the diagnosis and differential diagnosis, and gives an update on the emerging views on its pathogenesis, suggested new diagnostic MRI patterns, and other suggested biomarkers as well as management issues for the neurologic disease that include the use of anti-TNF-alpha agents.

Key points

 

• Behcet disease is a disorder of unclear pathogenesis. Several inflammatory markers and the association of Behcet disease with particular major histocompatibility complex antigens point to inflammatory mechanisms inducing the manifestations of the disease in a genetically predisposed host.

 

• Neurologic involvement, or Neuro-Behcet syndrome (NBS), is heterogeneous with 2 major clinical subtypes: a central nervous system inflammatory parenchymal disease and a less common nonparenchymal form that involves large extraparenchymal vascular structures, mainly venous dural sinuses. Dural venous sinus thrombosis is not uncommon in most cases of pediatric NBS. Peripheral nervous system involvement is extremely rare.

 

• NBS is a great mimicker of various central nervous system disorders. A history of oral or genital ulcers should be sought in any patient with brainstem and white matter lesions, increased intracranial pressure, or venous thrombosis.

 

• New anti-inflammatory agents, in particular TNF-alpha inhibitors, are likely to provide a more efficient therapeutic alternative to conventional immunosuppressive drugs.

Historical note and terminology

The earliest known observations of oral and genital ulcerations associated with uveitis are in Hippocrates' notes (Feigenbaum 1956). The disease was named after Dr. Hulusi Behcet (Behcet 1937).

Image: Dr. Hulusi Behcet
Since then, the manifestations of the disease have become more widely known and have been extended to include multiple systems.

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