Behçet Disease

  • Autoimmune disorder causing vasculitis principally in eyes, mucous membranes, skin, but also in many other organs
  • Diagnosed most commonly in Middle East and Asia around old Silk Road trading route
  • Main systemic manifestations: recurrent painful mouth and genital ulcers, arthritis, meningoencephalitis
  • Main ophthalmic manifestations: anterior uveitis often with hypopyon, retinal vasculitis, vitreous cells, optic neuropathy, papilledema from dural venous sinus thrombosis
  • Refer urgently to ophthalmologist any patient with diagnosis of Behçet disease who has newly blurred vision
  • Refer non-urgently any patient without visual symptoms in whom Behçet disease is suspected in order to help confirm diagnosis and detect manifestations of mild ophthalmic involvement
  • Diagnosis depends on combination of characteristic clinical features, exclusion of other diseases, and pathergy test, but...
  • Diagnosis always remains presumptive as clinical features, laboratory tests, pathology not specific
  • Treatment involves immune suppressants, including corticosteroids, cyclosporine, mycophenolate, azathioprine, cyclophosphamide, tumor necrosis factor inhibitors
  • Outcomes depend on severity of manifestations and promptness of diagnosis