Behçet syndrome: from pathogenesis to novel therapies

Clin Exp Med. 2016 Feb;16(1):1-12. doi: 10.1007/s10238-014-0328-z. Epub 2014 Dec 2.

Abstract

Behçet syndrome is a chronic disease hallmarked by inflammation of the blood vessels that is related to an autoimmune reaction caused by inherited susceptibility due to specific genes and environmental factors, probably components of infectious microorganisms, which turn on or get going the disease in genetically susceptible subjects. The more common clinical expression of the disease is represented by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis, sometimes associated with inflammatory arthritis, phlebitis, iritis, as well as inflammation of the digestive tract, brain, and spinal cord. The treatment strategies used to manage the manifestations of Behçet syndrome have gradually progressed, and a number of new therapeutic resources have been implemented in recent years, allowing better control of pathogenic mechanisms, reducing symptoms and suffering, and ameliorating patient's outcome.

Keywords: Aphthae; Arthritis; Behçet; Immunosuppression; Uveitis; Vasculitis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Anticoagulants / therapeutic use
  • Behcet Syndrome / drug therapy*
  • Behcet Syndrome / etiology
  • Behcet Syndrome / pathology*
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Treatment Outcome

Substances

  • Anticoagulants
  • Glucocorticoids
  • Immunosuppressive Agents