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Behcet's Syndrome

Turkish dermatologist, Hulusi Behcet (1889-1948) recognized and reported in 1937 symptoms of Behcet's syndrome. A dental infection is attributed as the etiology of the disease. The American Behcet's Disease Foundation (ABDA) was founded in 1978 with the objective to provide support to patients and family as well the caregivers. In the absence of a cure or a single test to definitely determine Behcet's syndrome or Behcet's disease, educate about the syndrome to seek prompt medical attention for treatment.


Morbus Behcet or Silk Road disease is the other name for Behcet Syndrome or disease. Month of May is Behcet's Awareness month and May 20th is Behcet's awareness day. The focus is on spreading awareness and stress the importance of self-help.


Behcet's syndrome facts


  • Non-contagious
  • Exact cause is unknown
  • Genetic predisposition is high.
  • Symptoms are similar to other disease of the digestive tract, Ulcerative Colitis and Crohn's Disease
  • No single test to confirm diagnosis.
  • No cure available.
  • Disease affects different parts of the body.
  • Men and women are equally prone but more severe in men.
  • In the US, affects more women.
  • More prevalent in Middle East, Mediterranean and Eastern Asia.
  • Turkey has the highest prevalence.
  • Leading cause of blindness in Japan.
  • Most common among age group 20-30.
  • Treatment requires more than one specialist.
  • Continued, extensive research to explore possible genetic, bacterial and viral cause.
  • Research is on to identify medicines to better treat Behcet's disease.

Behcet's syndrome – Autoimmune disease

There are over 14, 000 auto-immune diseases and 7000 plus are rare. Behcet's syndrome is rare. Autoimmune disease occurs when the body's immune system mistakenly attacks its own healthy tissues. Though the clinical feature of auto immunity is absent, Behcet's syndrome is classified as an autoimmune disease as it has various aspects related to autoimmune disease. Enhanced inflammatory response (inflammation of blood vessels) is one such aspect. Significant number of women as compared to men are more likely to be affected by autoimmune disease. Estrogen predisposes women to autoimmune disease.


Behcet's syndrome symptoms

The inflammation of blood vessels, particularly veins causes symptoms in many parts of the body. Swelling, redness, heat and pain are select features of an inflammation. Though Behcet's disease can affect any part of the body, involvement of the neurological system is known as neuro-Behcet's disease and is rated the most disabling complication of the disease. Not that common, neuro-Behcet's disease affects about 10 per cent of people with Behcet's disease.


The most common symptom of Behcet's syndrome is the regular occurrence of ulcers in the mouth and genitals. The symptoms are an off-shoot of inflammation of the eyes, skin, arteries, veins, joints, nervous and digestive systems and heart. Symptoms can be mild or severe. Symptoms can be noticed between ages 20-30 years.

Mouth ulcers: May look like normal mouth ulcers but are more painful and numerous in number. Ulcers develop in the tongue, lips, and gums and inside of the cheeks. Even if the ulcers heal within a couple of weeks, they recur.


Genital ulcers: In men, though genital ulcers can appear anywhere in the groin area, including the penis, it is more common on the scrotum. In women, the ulcers appear on the cervix (neck of the womb), vulva or vagina. The ulcers are usually painful and scars appear around the area.

Skin lesions: Resembling acne, pustular skin lesions can appear anywhere on the body. Erythema nodosum results in red, painful, tender lumps that can measure one to five centimeters. It is the result of inflammation in the fatty layer of the skin. It can appear on the legs and ankles but can also appear on the face, neck or arms. Erythema nodosum related to other disease heal without scars but if related to Behcet's disease, leaves the skin totally discolored.


Inflammation of the eyes: Sudden inflammation of the eyes is a common symptom of Behcet's syndrome. A group of connected tissues, uveal tract is inside the eye. This uveal tract gets inflamed. Uveitis as it is named can cause symptoms such as, painful red eyes, blurred vision and floaters (dots that move across the field of vision). With a possibility of permanent visual impairment, it is best to seek medical attention for appropriate treatment without any delay.

Skin sensitivity: Pathergy is a condition signifying sensitivity of the skin, particularly injury or irritation. Even a needle prick can lead to developing a large red lump in a day or two.


Gastrointestinal symptoms: Inflammation of the stomach and intestines causes symptoms such as loss of appetite, stomach pain, indigestion, diarrhea, feeling sick and vomiting. There is a possibility of damage to the bowel resulting in bleeding. Blood in stools suggests inflammation of the internal lining of the bowel.


Blood clots: Inflammation of the veins can lead to formation of blood clots or thrombosis.

Joint pain: Joint pain in ankles, wrists, knees, elbows and hips is common. Inflammation in the joint can cause swelling, redness and tenderness.

Brain: Meninges is the coverage of the brain. Inflammation of the brain or tissue that covers the brain (meninges) causes symptoms like headache, neck stiffness along with high body temperature. In severe cases, it can damage the nervous tissue and lead to extreme weakness or impaired function of the body.

Aneurysms: Aneurysms are outpouchings of blood vessel walls due to inflammation of arteries in the lungs. This can lead to massive lung hemorrhage. Symptoms include pain in the limbs, severe headache, feeling dizzy, and breathlessness, coughing up blood, confusion and loss of consciousness.

Inflammation of the nervous system: Inflammation of the central nervous system occurs in 5%-10% of reported cases. This is regarded as the most serious symptom. Typical symptoms include headache, double vision, loss of balance, seizures, partial paralysis on one side of the body, personality changes. Any of these are noticeable within the first five years of recognizing initial symptoms.

General symptoms: Experiencing extreme fatigue to the extent that it interrupts with everyday routine is a general symptom of Behcet's syndrome.


Behcet's Syndrome Causes

The exact cause remains unclear. Though regarded as an autoimmune disorder, it is unclear what triggers the autoimmune disorder. Other possible association is genetics. Certain ethnic groups or a family member with the disease increases chances of developing Behcet's syndrome. People with gene HLA B51, variations in other genes like IL10, IL23R-IL2RB2 increases the risk of developing the disease considerably. However, the condition does not have a clear pattern of incidence.


Risk factors identified include:

Age: Children and older adults can develop the condition. But men and women in the age group of 20-3 are more likely to be affected.

Location: People from countries in the Middle East and Far East, including Turkey, Iran, Japan and China are more likely to develop Behcet's syndrome.

Sex: The disease is more severe in men.

Genes: Having certain genes increases the risk of developing the syndrome.


Behcet's Syndrome Diagnosis

In the absence of a single test to diagnose Behcet's syndrome, doctors look out for symptoms. Blood tests or other laboratory tests are recommended to rule out other disease or illness. The criteria include:

Mouth sores: Mouth sores are very common. Many disorders are related to mouth sores. If the mouth sores recur every three months in 12 months, it is regarded as criteria for evaluating the disease.

Doctors look for two additional signs.



Behcet's Syndrome - time to seek help

Recognizing symptoms and relating the symptoms to Behcet's disease is essential. Note down symptoms being experienced and add related information that affects normal routine. Seek appointment with a doctor. If medical attention is delayed, the condition can worsen and lead to losing eye sight or a stroke. At the meeting, divulge family history and inform about medications for any other health issue or supplements being taken.


You are most likely to seek specialist help. Rheumatologist for arthritis, joint pain etc, Ophthalmologist for eye problems, gynecologist or an urologist for genital sores, dermatologist for skin issues, Gastroenterologist for digestive difficulties or neurologist for symptoms related to CNS (central nervous system).


Behcet's Syndrome Treatment

There is no cure for Behcet's syndrome. The treatment is aimed at alleviating the symptoms, reducing the frequency and intensity, put the disease into remission and prevent serious complications. Medicines for controlling the individual symptoms are prescribed. Some medicines are prescribed with other medicines to suppress the activity of immune system. Some medicines can have side effects too.

For the skin: gels, creams and ointment that contain a Corticosteroid to reduce the inflammation.


For mouth sores: Special mouth washes with Corticosteroid to reduce the pain and associated discomfort.

For the eyes: Eye drops with Corticosteroid to relieve pain and redness in the eye.


Coping with Behcet's syndrome


  • Take ample rest in between flares.
  • Pay attention to diet and exercise.
  • Find support groups and connect.
  • Login to American Behcet's Disease Association.
  • Read message boards and engage in chat sessions.

Scleritis

Scleritis is a serious eye disease that refers to the inflammation of the sclera, the white outer portion of the eye. Sclera is made up of connective tissues and thus helps in protecting the eye. Sclera is also responsible for giving spherical shape to our eyes. When sclera develops inflammation it is termed as scleritis.

Normally scleritis occurs between the age of 30 and 60 and also scleritis affects women more than men. Scleritis is potentially damaging and may even cause permanent vision loss in severe cases. Most often scleritis is associated with other underlying auto immune diseases present in the body such as rheumatoid arthritis, Gout, Wegener granulomatosis to name a few. But in few cases the disease may occur without any underlying condition and in such instances, the cause remains unknown. Scleritis can occur in the front portion of the eye ball or the back part of the eye ball and they are known as anterior scleritis and posterior scleritis respectively. Anterior scleritis is further classified into Nodular scleritis, Diffuse scleritis, Necrotizing scleritis with inflammation and Necrotizing scleritis without inflammation.

In Nodular scleritis, tiny tender nodules form on the white portion of the eye. Diffuse scleritis refers to the inflammation of the front half of the sclera. This is the most common type of scleritis compared to other variations. Necrotizing scleritis is a serious form of scleritis and may even lead to vision loss. It is accompanied by severe pain and usually associated with the problem in other organs of the body. The sclera of the eye thins down severely and may occasionally result in perforation of the eye globe. This condition may surface with or without inflammation. Posterior scleritis is the rarest form of scleritis and usually presents itself with the symptoms like double vision, severe pain, retinal detachment, proptosis and restricted eye movement.


Diagnosis of Scleritis

Clinical examination of the eye is the first step towards assessing the scleritis disease. Further some eye tests and blood tests may be conducted to assess the severity and to diagnose the underlying disease that is causing the inflammation. Diagnostic procedures like ultrasonography and MRI may be advised if posterior scleritis is suspected.

Treatment of Scleritis

Firstly, oral anti inflammatory drugs are prescribed to control the inflammation and relieve the patient of the pain. In case of scleritis, topical eye drops alone are not sufficient to cure the inflammation. The main objective of the treatment would be to diagnose and treat the underlying disease that is causing the condition. In chronic cases, graft surgery may be performed to treat the injured portion of the eye.

Scleritis is treatable, but there are chances of this condition recurring again. The success rate of the treatment also depends upon the severity and the type of the scleritis. Necrotizing scleritis has low success rate and the incidence of vision loss is higher with this condition where as diffuse and nodular scleritis are easily treatable.


Colitis

An inflammation of the inner lining of the colon is colitis. Colitis is an inflammation in the colon and can be associated with diarrhea which could be due to infection, loss of blood supply to the colon, inflammatory bowel disease and invasion of the colon wall with lymphocytic white blood cells or collagen. While the many causes of colitis may be self-limiting and can be treated with diet and observation, nevertheless it is important to find why the inflammation had occurred as there can be more serious causes.


In case of ulcerative colitis, the exact cause remains unknown and previous diet and stress are suspected. However, doctors opine that these causes may aggravate but not cause ulcerative colitis. One possible reason could be immune system malfunction. An abnormal immune response can cause the immune system to attack the cells in the digestive tract and thereby cause malfunction. Heredity can also play a role in ulcerative colitis and it is more common in those who have a family history of the disease.


Infectious Colitis

The most common bacteria that can cause colitis are Campylobacter, Shigella, E. coli, Yersinia, and Salmonella. Most of the time infections occur as the patient has eaten contaminated food. Hence the symptoms of diarrhea with or without blood, abdominal cramps, dehydration from loss of water.

Yet another bacteria that can cause colitis is Clostridium difficile. While C.diff which is found in the colon of a healthy individual co-exists with other normal bacteria, when antibiotics are prescribed, the susceptible bacteria in the colon can be destroyed and this allows clostridia to grow unchecked, and thereby cause colitis. Unfortunately, these bacteria are also found in many surfaces in the hospitals, and hence can be contagious from person to person.


In fact, worldwide the most common parasite infection that can cause colitis is Entamoeba histolytica. This is acquired by drinking infected water and can also be passed on from one to another because of poor hygienic and sanitation conditions.

Microscopic colitis can infiltrate the wall of the colon, as a result of inflammation and this is an uncommon illness and could be an auto-immune disease. In this case, the diarrhea is watery and no blood is present in the feces. In infants younger than a year, colitis is often due to allergies to cow and soy milk. Allergic colitis can also be seen in breast fed babies, where the mother drinks cow's milk and passes that protein into their breast milk.


Ulcerative Colitis can be severe depending upon its location, reading from the signs and symptoms. Depending upon which part of the colon is inflamed it could cause diarrhea, abdominal cramps, rectal pain, rectal bleeding, urgency to defecate, inability to defecate despite urgency, weight loss and fatigue, fever and failure to grow in children.


Diagnosis of Colitis

To confirm the diagnosis, the doctor may prescribe the following tests and procedures:


  • Blood tests for anemia and to check signs of infection.
  • A stool sample to help rule out other disorders such as infections caused by bacteria, viruses and parasites.
  • Colonoscopy to view the entire colon. The doctor can take a small biopsy during the procedure for laboratory analysis. A tissue sample can help confirm the diagnosis.
  • Flexible Sigmoidoscopy to examine the last portion of the colon and if it is severely inflamed, the doctor can perform this test instead of colonoscopy.
  • X rays of abdominal area to rule out serious complications, such as a perforated colon.

Treatment

There are several categories of drugs depending upon the severity of the condition. The drugs may work well for some but not for all. Also, drugs can cause serious side effects which can far overweigh the benefits of the treatment.

Anti-inflammatory drugs are the first step in the treatment of inflammatory bowel disease. Aminosalicylates, and Corticosteroids and immune system suppressors are prescribed. These are given orally, intravenously or by enema depending upon the location that is affected. Corticosteroids can have several side effects including puffy face, excessive facial hair, insomnia and hyperactivity and more serious ones such as hypertension, diabetes, osteoporosis, bone fractures and cataracts. These are not usually prescribed for long term.

While immune system suppressors can reduce inflammation, they do so by suppressing the immune system and therefore start the process of inflammation. A combination of drugs works better is most cases. Other medications include antibiotics, anti-diarrheal medications, pain relievers and iron supplements.


Surgery

Surgery can eliminate ulcerative colitis by removing the entire colon and rectum. This procedure called ileoanal anastomosis eliminates the need to wear a bag to collect the stool. The surgeon constructs a pouch from the end of the small intestine. This pouch is then attached directly to the anus allowing expulsion of waste relatively normally. When a pouch is not possible, the surgeon creates a permanent opening in the abdomen, through which the stool is passed for collection into an attached bag.


Complications of colitis

It is recommended to have more frequent screening for colon cancer because of increased risk although it will depend on the location and duration of the disease. Some complications that can occur:


  • Severe bleeding
  • Perforated colon
  • Severe dehydration
  • Liver disease
  • Osteoporosis
  • Inflammation of joints, skin, joints of eyes, and sores in the lining of mouth
  • Increased risk of colon cancer
  • Swelling colon
  • Increased blood clots in veins and arteries.

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Bibliography / Reference

Collection of Pages - Last revised Date: November 17, 2019