[Adamantiades-Behçet's disease. Clinical review].
Behçet's disease is a chronic relapsing systemic vasculitis of unknown etiology, affecting predominantly oral and genital mucocutaneous tissues and also the eyes. The disease is spread worldwide with a higher prevalence rate in countries along the ancient Silk Route, but it is rare (1-10/100,000) in Central and Northern Europe. Genetic, environmental, immunologic, inflammatory and rheologic factors are involved in the pathogenesis and the course of the disease. Any vascularized organ may be affected. Eye involvement is frequent, and may eventually result in loss of vision. Further important complications are cerebral manifestations, thrombotic syndromes, and arterial aneurysms with a high risk of rupture. Diagnosis and therapy of Behçet's disease are best managed by an interdisciplinary team. Skin lesions may be controlled by systemic treatment with colchicine, alternatively with dapsone, and in severe cases with thalidomide. Active systemic disease should be treated more aggressively using immunosuppressants. Despite advances in treatment relapses are still frequent, and systemic disease remains associated with an adverse prognosis.
Störk
Stefan
S
Medizinische Klinik und Poliklinik I, Universität Würzburg, Klinikstrasse 6-8, Würzburg. stoerk_s@klinik.uni-wuerzburg.de
Kneitz
Christian
C
Bröcker
Eva-B
EB
Hoyer
Caroline
C
Ertl
Georg
G
Angermann
Christiane E
CE
D000328:Adult; D000367:Age Factors; D001528:Behcet Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D002648:Child; D003078:Colchicine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic; D012008:Recurrence; D013792:Thalidomide / Q000627:therapeutic use
D007166:Immunosuppressive Agents; D013792:Thalidomide; D003078:Colchicine