Behcet’s Disease

Behcet’s Disease

Sometimes called Behçet’s syndrome, Morbus Behçet, Behçet-Adamantiades syndrome or Silk Road disease, is a rare immune-mediated small-vessel systemic vasculitis that often presents with mucous membrane ulceration and ocular problems. Behçet’s disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. As a systemic disease, it can also involve visceral organs such as the gastrointestinal tract, pulmonary, musculoskeletal, cardiovascular and neurological systems. This syndrome can be fatal due to ruptured vascular aneurysms or severe neurological complications.

Symptoms

·         Skin irritation

·         Hereditary

·         Arthritis/Arthralgia

·         Bowels – GI manifestations include abdominal pain, nausea, and diarrhea with or without blood, and they often involve the ileocecal valve. Many patients with BD often complain about abdominal tenderness, bloating, and generic abdominal discomfort that closely mimics irritable bowel syndrome.

·         Cardiac – Pericarditis is a frequent cardiac manifestation.

·         Cardio-Vascular Problems Of An Inflammatory Origin

·         Changes Of Personality, Psychoses

·         Chronic aortic regurgitation due to aortic root disease may also be seen

·         Deep Vein Thrombosis

·         Epididymitis

·         Extreme Exhaustion

·         Inflammatory Problems In Chest And Lungs

·         Lungs – Lung involvement is typically in the form of hemoptysis, pleuritis, cough, or fever, and in severe cases can be life-threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs

·         Mouth Ulcers

·         Musculoskeletal system – Arthralgia is seen in up to half of patients, and is usually a non-erosive poly or oligoarthritis primarily of the large joints of the lower extremities.

·         Nearly all patients present with some form of painful oral mucocutaneous ulcerations in the form of aphthous ulcers or non-scarring oral lesions.

·         Nervous System Symptoms

·         Neurological involvements range from aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss

·         Neurological system – CNS involvement most often occurs as a chronic meningoencephalitis. Lesions tend to occur in the brainstem, the basal ganglia and deep hemispheric white matter and may resemble those of MS. Brainstem atrophy is seen in chronic cases.

·         Ocular system – Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20% of cases.

·         Painful genital ulcerations usually develop around the anus, vulva, or scrotum and cause scarring in 75% of the patients.

·         Problems With Hearing And/Or Balance

·         Stomach And/Or Bowel Inflammation

·         Superficial Thrombophlebitis

·         The oral lesions are similar to those found in inflammatory bowel disease and can be relapsing.

 

 

Resources

Resources

Treatments

  • Anti-TNF agent, etanercept, may be useful in people with mainly skin and mucosal symptoms
  • Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease.
  • Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. The quality of the evidence for treating the oral ulcers associated with Behcet’s, however, is poor
  • Dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions
  • Early identification and treatment is essential.
  • Given its rarity, the optimal treatment for acute optic neuropathy in Behçet’s disease has not been established.
  • High dose corticosteroid therapy is often used for severe disease manifestations.
  • Immunosuppressants such as interferon alpha and tumour necrosis factor antagonists may improve though not completely reverse symptoms of ocular Behçet’s, which may progress over time despite treatment.
  • Interferon alpha-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers as well as ocular lesions. Azathioprine, when used in combination with interferon alpha-2b also shows promise, and Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis
  • IVIG could be a treatment for severe or complicated cases
  • Lumbar puncture or surgical treatment may be required to prevent optic atrophy in cases of intracranial hypertension refractory to treatment with immunomodulators and steroids.
  • Response to ciclosporin, periocular triamcinolone, and IV methylprednisone followed by oral prednisone has been reported although relapses leading to irreversible visual loss may occur even with treatment
  • Secondary optic nerve atrophy is frequently irreversible.
  • Thalidomide has also been used due to its immune-modifying effect.
  • When symptoms are limited to the anterior chamber of the eye prognosis is improved. Posterior involvement, particularly optic nerve involvement is a poor prognostic indicator.
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