Behçet's Basics

The information in this section contains excerpted material
from my book Essential Guide to Behcet's Disease, as well as
information from other medical books/ journals, the American
Behçet's Disease Association, physicians and other patients.
All information on this web site, and in Behçet's Basics, is
© 2000-2008 by Joanne Zeis, and was last updated on 5/6/08.
Many thanks to Lisa Jensen, MPH, past Vice President/Board
of Directors of the ABDA for her careful review of the original
"Basic Information on Behcet's" booklet's contents. 

I am not medically trained. Please do not initiate or change any 
treatment plan on your own. If you have any questions after reading 
this material, please meet with your primary care physician or 
health care provider. You may, however, e-mail me with 
general questions by clicking on this link.

Subjects covered in this section: (click on a link to advance to that topic, or scroll down)

What is Behçet's disease?
How is Behçet's diagnosed?
The symptoms of Behçet's disease (International
     Classification Criteria of 1989)
What causes Behçet's, and how will it affect my
     family and my life?
What is it like after 29 years of symptoms?

What is Behçet's disease?

Behçet's disease is a rare, multi-system rheumatic disorder that causes the inflammation of blood vessels anywhere in the body. This inflammation is known as vasculitis. The location of the inflammation(s) is what dictates your symptoms.   

There are many different pronunciations of the word "Behçet's". Options include the following: Beh SHETS, Beh SHAY and Beh CHETs (which is the correct Turkish pronunciation, although rarely used in the U.S.).

Behçet's disease was named for Hulusi Behcet, a Turkish doctor who first identified this “triple-symptom complex.” He published a paper in 1937 noting “recurrent oral and genital ulcerations, as well as hypopyon-iritis” in two of his patients. We now know that Behçet's disease (BD) encompasses a much wider range of medical problems beyond these three classic Behçet's symptoms. 

Behçet's disease tends to strike people with “Silk Road” bloodlines more frequently than it affects people of other ancestries. Silk Road countries include those in the Mediterranean basin, Middle East and Far East. However, cases of Behçet's disease (or syndrome) have been found world-wide, regardless of patients’ backgrounds. Therefore, a Behçet's diagnosis should not be ruled out in anyone who displays the primary symptoms described in the International Criteria for Behçet's Disease -- even if the patient doesn’t have a traditional Silk Road heritage. 

In the Silk Road countries, Behçet's disease is more common in men than in women. However, in Western Europe and the U.S., the trend is reversed: more women have BD than men. Symptoms typically develop when patients are in their 20s and 30s, but the disease has been seen in all ages, from infants to the elderly.  

There are approximately 15,000 people with Behçet's disease in the U.S. 

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How is Behçet's diagnosed?

The diagnosis of Behçet's disease is based on having a specific set of symptoms. Contrary to what some health care providers say, it’s not necessary to show all of the diagnosable medical problems of BD at the same time, in one office visit. Unfortunately, there is no test available right now that will definitely diagnose a case of Behcet's disease.

Behçet's may start with just one or two small symptoms that come and go -- typically, ulcerations -- and then other symptoms or clues may appear gradually over the years. Because BD can affect so many different body systems, patients usually see a variety of specialists, none of whom may be aware of the “big picture” at first. As a result, it might actually take several years before all of the clues are put together and a diagnosis of Behçet's is reached. That’s why medical documentation of your symptoms, as they appear, is very important. In the event that you can’t be seen by a doctor before your symptoms fade, personal documentation (for example, pictures of skin lesions, or a daily record of health problems) can be helpful for future reference.

Some of the symptoms of Behçet's disease also appear in other diseases such as lupus, Lyme disease and Crohn’s disease, to name a few. It may be necessary to undergo various blood tests and/or biopsies in order to rule out these other diseases before you can be diagnosed with Behcet’s.

Being diagnosed with Behçet's disease can cause a lot of worries and frustration -- worries because you don’t know what the future will bring, and frustration because, even with a diagnosis, you’ll come up against specialists who will say that your symptoms are all in your head. You may have heard this line of thought already, from more than one doctor. Of course, you know that your medical issues are real.

According to the International Criteria for Classification of Behçet’s Disease (est. in 1989), these are the primary symptoms of Behçet’s disease (notes in brackets [ ] are my own, and not part of the criteria):

•     recurrent oral ulcerations in almost 100% of patients 
      [painful aphthous ulcers, testing negative for herpes]

    plus any two of the following four symptoms:

•     recurrent genital ulcerations [resembling oral ulcers, 
      usually painful, testing negative for herpes]

•     eye inflammation -- uveitis (anterior or posterior),
      cells in the vitreous, or retinal vasculitis

•     skin lesions (erythema nodosum, pseudo-folliculitis, 
      papulopustular lesions or acneiform nodules
      consistent with Behçet's disease, observed by a
      physician in post-adolescent patients not receiving
      corticosteroids)

•     a positive pathergy test [a special skinprick test using
      a sterile needle and sterile saline solution, performed
      during a  time of active Behçet's symptoms. However,
      North American patients rarely test positive, even
      during disease activity.]

IMPORTANT: The International Criteria show the symptoms that are most likely to indicate a case of Behcet's, in the absence of any other medical explanations. The originators recommended that the symptom list be known as "classification"  criteria rather than "diagnostic" criteria -- they felt that the list was more useful as a way to group patients for Behcet's research studies, rather than as a way to diagnose individual cases of BD. However, because of a lack of laboratory tests specific for Behcet's, and with limited clinical experience in treating BD patients, many physicians lean heavily on the International Criteria to help with diagnosis. Unfortunately, using the criteria alone may keep some patients from being correctly diagnosed. Readers should know that there are at least six other Behcet's criteria lists in existence, originating from various countries around the world. A patient may meet the criteria from one country's list, but not from another. Therefore, a physician's experience in treating previous Behcet's patients is essential to receiving a proper diagnosis.

Other symptoms that may be useful in diagnosis, but are not considered part of the International Behçet’s Criteria:

•     subcutaneous thrombophlebitis [inflammation of a
      vein beneath the skin]

•     deep vein thrombosis [development of a blood clot]

•     epididymitis [swelling and pain in one or both
      testicles]

•     arterial occlusion and/or aneurysm [stroke]

•     central nervous system involvement [e.g.: difficulties
      in movement or speech, memory loss]

•     severe headaches with stiff neck [possible aseptic 
      meningitis]

•     joint pain [arthralgia] or non-destructive arthritis 

•     GI tract involvement [e.g.: bloating, cramping,
      diarrhea, bloody stools. Lesions may appear
      anywhere in digestive tract, from mouth to lower
      bowel]

•     renal involvement [kidney function abnormalities]

•     pulmonary [lung] vascular inflammation and pleuritis

•     family history

Along with the symptoms described above, you may experience severe fatigue -- although as a general rule, fatigue can be a problem in many of the autoimmune diseases. It can also be caused by some medications.

Many patients have questioned whether Behçet’s disease can create problems with their hearing -- for example, tinnitus, fluctuating hearing loss, sudden deafness, and/or dizziness from inner ear vasculitis. For more information on this subject, please refer to the following journal articles:

•     Gemignani G, Berrettini S, et al; Hearing and
      vestibular disturbances in Behçet’s syndrome;
      Ann Otol Rhinol Laryngol 1991; 100:459-463.
   

•     Soylu L, Aydogan B, Soylu M, Ozsahinoglu C;
      Hearing loss in Behçet’s disease; Ann Otol Rhinol
      Laryngol 1995 Nov;104: 864-867.

It is also possible to have cardiac disturbances associated with Behçet’s disease. This includes arrhythmias (abnormally slow or fast heartbeats). For more information on cardiovascular problems, please see the following reference:

•     Morelli S, Perrone C, Ferrante L, Sgreccia A, Priori
      R, Voci P, et al. Cardiac involvement in Behçet's 
      disease;Cardiology.1997 Nov-Dec;88(6):513-7.

Some physicians like to divide their Behçet’s cases into "Complete" versus "Incomplete" categories. The criteria used for these categories can differ from physician to physician. In general, though, a patient with "Complete" Behçet's has three or more of the major symptoms described in the International Criteria, and one or more of the minor symptoms. A patient with "Incomplete" Behçet's has at least two major symptoms, and one or two minor symptoms.

Patients with Incomplete BD may or may not progress to a full-blown case of Behçet's.

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What causes Behçet's, and how will it affect my family and my life?

There is no known cause for Behçet's disease, although some people suspect that an environmental element, an unknown virus, or perhaps a bacterial infection may trigger the disease in people who already have a genetic predisposition for Behcet’s. While there is no cure for BD at the present time, there are many medications available to help with the various symptoms.

Behçet's isn’t contagious. While it’s possible that someone you know may show some of your same symptoms, their symptoms may be caused by a medical problem totally unrelated to BD. There are no guarantees that any of them will eventually develop Behcet’s. This includes any children you may have, now or in the future. Autoimmune diseases of all sorts can run in families, though, so you might find that while you have Behcet’s, you may have a couple of other relatives with diseases like lupus or ulcerative colitis instead.

Some newly-diagnosed people are worried because they think that having Behçet's means they can no longer have a normal life. Granted, your life may now be different from what it was, but many patients are still able to carry on with family, friends and work -- just at a reduced level, depending on their symptoms.

Behçet's is a chronic, unpredictable disease, and there’s no definite progression of symptoms. It causes your immune system to be overactive, so check with your doctor before taking any herbal remedy (such as echinacea) to boost your immune system, because it may make your symptoms worse. Some people never develop serious eye disease or have nervous system involvement. For most people, Behçet's is a long-term, cyclical disease that comes and goes in “flares” of varying intensities. You may have symptom-free periods of weeks or months that are interrupted by flares that can last a few days, weeks, or months. You may have awful symptoms right now, and find that you have different (or many less) symptoms during the next flare. Some people can be hospitalized at times with the more serious complications of BD, but there are also occasional reports of people who go into permanent remission. As you can see, it’s all very uncertain. The only constant is that most Behçet's patients deal with some level of symptoms for their entire lives.  

At the present time, there is no way to predict which patients will move on to more serious problems, which ones will remain stable, and which ones might go into remission. 

Learn to listen to your body, and recognize the signs of an oncoming flare. If you start to feel ill, slow down your activity level if possible, and be kind to yourself when making plans. Realize that emotional and physical stress sets off flares in many patients. It may not matter whether the stress is bad (a death in the family) or good (getting married) -- it may still have the same overall effect on your health. Some patients report that severe cold or high heat and humidity can initiate health problems, or that certain seasons are more difficult on their bodies than other times of the year. Women may notice an increase in their symptoms immediately before or during their periods. 

Whether or not to have children is a highly personal issue for each couple when one partner has Behçet's disease. Couples justifiably wonder if their child(ren) will eventually develop BD. Just as in the general population, though, any of your children may grow up strong and healthy, or they may have a tendency towards illness of some sort -- and that illness may or may not be Behçet's. At the present time, there is no way to predict the final outcome. There can also be natural concerns about how the stress of pregnancy will affect a woman's illness. Some women report that their symptoms stopped completely during their pregnancies, while other women were plagued by an increase in their BD-related problems. Any woman considering pregnancy should thoroughly discuss her concerns with her healthcare practitioner in advance -- including which of her medications she may or may not continue to take during her pregnancy. I conducted a study in 2006 of pregnancy in U.S. women with Behcet's disease, and the results were presented at the International Conference on Behcet's Disease in Portugal in Sept 2006. Please contact me for more information about the results.

Because so few people have Behcet’s, it’s common to feel alone in your problems. Not many people are able to relate to your health issues because they’re so rare. You may find that you have difficulties with the significant people in your life because they can’t understand what’s wrong with you -- after all, you look “healthy” most of the time. Of course, most of the problems caused by Behçet's inflammations are internal, so they’re not immediately apparent to your friends and family. It’s common to have days when you feel ill, but look fine on the outside.  

If you find yourself becoming depressed by your health issues or worried about your future, please seek out a counselor or therapist who will listen and help. In addition, there are Behçet's support groups available on the internet, with members who are dealing with the very same issues that you face on a daily basis. A list of available support groups appears on the Behçet's Links page.

The American Behçet's Disease Association (PO Box 869, Smithtown, NY 11787-0869; phone 800-723-4238) publishes a newsletter for ABDA members and has an informational website at www.behcets.com  that you might find helpful. The ABDA also runs an online support bulletin board for its members, and provides a Doctor Registry with names/locations of physicians familiar with treating BD in the U.S. The most recent ABDA Patient & Medical Conference took place in Las Vegas in April 2007, and featured presentations and question/answer sessions run by physicians familiar with Behçet's disease. There were also formal and informal patient and family discussion groups throughout the weekend. In addition to the Conference, the ABDA sponsors a "Behcet's Walk" fundraiser for patients, families and loved ones on Long Island (NY) every 2-3 years. The next Walk will take place on October 18, 2008.

The Twelfth International Conference on Behcet's Disease took place in Lisbon, Portugal from September 20-23, 2006. The next International Patients' and Medical Conference will take place in Austria from May 24-27, 2008. More information is available at: http://www.sambax.com/icbd

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          What is it like after 29 years of symptoms?

Because every person is different, not every patient will display the same symptoms or disease progression. After 29 years, some people may find their symptoms have lessened, and that some problems have disappeared entirely. For other people, their health issues may have become physically or emotionally disabling. My 29-year history is a little better than average in terms of symptoms and complications. I’ve rarely had the overwhelming fatigue or severe headaches experienced by many other patients. I still have good vision, although constant eye inflammation during the first 10 years took its toll. There has been short-term blindness in one eye due to a vitreous hemorrhage, right-sided blindness from a cataract, and now slightly distorted vision in both eyes from various retinal complications. Vision-related procedures have included cryosurgery, laser surgeries, removal of cataracts, and an IOL implantation in each eye. In spite of all of these problems, exceptional medical treatment has brought my vision back to 20/30. If you need to find an eye specialist experienced in treating visual complications of Behcet's such as uveitis, please refer to the following list:
http://uveitis.org/patient/specialists/default.html

My mouth ulcers used to appear in crops of up to 100 at a time, although colchicine reduced the outbreaks significantly. There has been minor neurological involvement, but no permanent impairment in my movement or speech. There has also been occasionally severe joint pain, peripheral neuropathy (tingling, pain and numbness in hands and feet), skin lesions and genital sores.

However, I'm a married mother of two healthy children, I work full-time as a medical writer, I play piano in the music ministry of my church, and I try to walk a mile a day. I realize that not everyone is able to maintain my level of activity, but I like to think that it helps keep me focused on my life, instead of my disease.

 

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© 2000-2008 by Joanne Zeis
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