Introduction Epidemiology Mucocutaneous Manifestations  Ocular Manifestations Major Vessel Involvement Central Nervous System Disease/ Gastrointestinal Lesions/ Arthritis and Spondylitis  Miscellaneous Manifestations/ Pathogenesis/ Pathology  Laboratory Findings/ Diagnosis/ Disease Activity  Management  Future Directions  References Editorial Board Contact Us Sign Up Archives

BEHÇET'S DISEASE

Kenneth T. Calamia, MD 
Division of Rheumatology 
Mayo Clinic 
Jacksonville, FL

Mehrdad Mazlumzadeh, MD 
Division of Rheumatology 
Mayo Clinic 
Rochester, MN

Central Nervous System Disease

Central nervous system symptoms in Behçet's disease may be due to aseptic meningitis or focal vascular lesions. Focal or multifocal nervous system involvement has a predilection for brain stem and periventricular white matter involvement. These lesions are best demonstrated on MRI. Although the findings are non-specific, a combination of stroke and aseptic meningitis in the presence of mucocutaneous lesions can be diagnostic. Cerebral angiography is usually negative because small vessels are involved. MR venography should be done in patients with papilledema or other symptoms suggesting increased intracranial pressure to rule out venous sinus thrombosis. Isolated headaches in Behçet's patients are common and may represent secondary migraine or be unrelated to the disease.

Gastrointestinal Lesions

Gastrointestinal symptoms include melena or abdominal pain. Lesions consist of single or multiple ulcerative lesions that primarily involve the distal ileum and cecum. Gastrointestinal lesions have a tendency to perforate or bleed and may recur after surgery. Vasculitis may be demonstrated in surgical specimens of operated patients. Lesions should be distinguished from those of Crohn's disease or those due to the use of non-steroidal anti-inflammatory drugs. As in Crohns, anti-Saccharomyces antibodies may be found in Behçet patients (12).

Arthritis and Spondylitis 

An intermittent, symmetric arthritis of the knees, ankles, hands, and/or wrists, affects 40% to 70% of patients with Behçet's disease (13). Episodes usually persist for a few weeks. Synovial fluid analysis reveals white blood cell counts >2000, consisting primarily of polymorphonuclear leukocytes. Synovial biopsies may reveal neutrophilic infiltration, unlike the lymphocytic synovitis seen in rheumatoid arthritis, and a destructive arthropathy is unusual. Ankylosing spondylitis may be found in Behçet's patients who are HLA-B27 positive. Fibromyalgia occurred in 16% of Turkish patients (14).