What is behcet syndrome ?

 BEHCET SYNDROME :

Behcet syndrome can create issues in your  mouth and genital area , in the form of recurrent sores . This condition is mostly associated with inflammation of both the blood vessels and the eyes , though it often causes a lot of different  issues throughout the body . Behcet disease , also known as Silk Road disease , is a chronic inflammatory condition, caused by vasculitis , which will affect and damage both your arteries and veins . The main common symptoms include painful sores on the muscous membranes of the mouth and other parts of the body, inflammation of parts of the eye , and arthritis . The sores can last from  a few days , up to a week or more . Often , the symptoms come and go . Diagnosis is based  on at least 3 episode of mouth  sores in a year  together with at least 2 of the following : genital sores, eye inflammation , skin sores, a positive skinprick test .



 WHAT ARE THE CAUSES  OF  BEHCET'S SYNDROME ?

It may be an autoimmune disorder, meaning the body's immune system mistaken  attacks healthy tissue with an inflammatory response . But ther are some people who have this gene marker who don't have Behcet disease . Researchers are studying other genes related to immune  function and think that infection may play a role in triggering the disease in some people who have this gene marker have BEHCET'S DISEASE . Researchers are studying other genes related  to immune  function and think that infections  may play a role in triggering the disease in some people  who have genetic markers that predispose them to Behcet' s. 



WHAT ARE THE SIGN & SYMPTOMS  OF BEHCET SYNDROME  ?



1. SKIN AND MUCOSA : Nearly all people with behcet disease present with some form of painful ulcerations inside the mouth . They are  a form of aphthouss ulcers or non - scarring oral lesions . The oral lesion are similar to  those found in inflammatory bowel disease and can be relapsing . Painful gential usually develop around the anus , vulva , or scrotum and cause scarring 75 % of the patients . Patients may present with erythema nodosum . cutaneous pustular vasculities , and lesions  similar to pyoderma . 

2. EYES : Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20 % of cases . Ocular involvement  can be in the form of posterior uveitis , anterior uveitis , or retinal vasculities . A rare form of ocular involvement in this syndrome is retinal vasulitis which presents with painless decrease of vision with the possibility of floaters or visual feild defects . 

Signs and symptoms of acute optic neuropathy include painless loss of vision  which may affect either one or both eyes , reduced visual acuity , reduced color vision . swollen optic disc. Progressive  optic atrophy may result  in decreased  visua; acuity or color vision . 

3. BOWELS : Some patients with BD experience abdominal tenderness , bloating  and general abdominal discomfort . When  mild  this can resemble irritable bowel syndrome , more severe cases bear similarities to inflammatory bowel disease  such as ulcertive colitis or crohn's .

4. LUNGS : Lungs 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  vasular  collapse  and death from bleeding in the lungs . 

5. JOINTS : Arthritis is seen in up to half of people , and is usually a non-erosive poly or  oligoarthritis primarily of the large joints of thr lower extremitis . 

6. HEART : Chronic  aortic regurgitation due to aortic root disease may also be seen . 

7. BLOOD VESSELS : Blood  vessel problems  are observed in 7-29 of people with arterial lesion representing 15 % of vasular lesions . Arterial lesion pose a greater risk . Most common arterial lesion are occlusion or stenosis and aneurysms or pseudoaneurysms.

HOW TO TREAT  BEHCET SYNDROME ?



Current treatment is aimed at easing the symptoms , reducing inflammation , and controlling the immune system . The quality of the evidence for treating the ulcers associated with Behcet disease , however is poor . 

High dose corticosteroid therapy is often used for severe disease manifestation . Anti TNF therapy such as infliximab has shown promise in treating  the uveitis associated with the disease . Apremilast may aslo be used to treat oral ulcers associated with behcet's disease.  Thalidomide has alos been used due to its immune - modifying  effect . in small studies  to have beneficial  results  for mucocutaneous lesions . 

The optimal treatment for acute optic neuropathy in  behcet 's disease has not been used to its immune modifying effect Immunosuppressants such as intereron alpha and tumor necrosis factor antagonists may improve though not completely reverse  symptoms of ocular behcet disease , which may  progress  over time  despite treatment . when symptoms are limited to the anterior   chamber of the eye prognosis is improved . Secondary optic nerve atrophy is frequently  irreversible . 

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