10 Interesting Facts About Behcet’s Disease

Behcet’s disease can be a lot of things: painful, embarrassing, confusing, frustrating, depressing, debilitating and, in rare cases, even fatal. But it can also be really, really interesting.

In this article, we’re going to take a look at 10 interesting facts about Behcet’s disease. But before we do, let’s briefly explore what Behcet’s is.

What Is Behcet’s Disease?

It’s a rare, chronic (long-lasting) illness that can affect multiple parts of the body.¹ Sometimes it’s classified as an autoimmune disease and sometimes an autoinflammatory condition, as Behcet’s seems to fit in somewhere between the two.²

Behcet’s disease is one of several types of vasculitis, which means blood-vessel inflammation.³ Behcet’s can cause inflammation in the eyes, skin, sinuses, brain, joints, kidneys, lungs, stomach, ears, and anywhere else in the body where there are blood vessels – which is pretty much everywhere.⁴

Symptoms of Behcet’s are often painful and can occasionally be quite serious. Common symptoms include oral ulcers, genital sores, uveitis (eye inflammation), arthritis (joint inflammation), fatigue, and a variety of skin problems. More serious symptoms of Behcet’s can include thromboses (blood clots), brain swelling, other nervous system problems, gastrointestinal (stomach, intestines, etc.) inflammation, blindness, hearing loss, and even death (rarely).⁵

That’s just a brief Behcet’s disease overview. If you’d like to learn more about what it’s like living with Behcet’s, you may want to read this: What Is It Like Having Behcet’s Disease? Now, let’s get to our 10 interesting facts about Behcet’s disease.

10 Interesting Facts About Behcet’s Disease

As much as it sucks being diagnosed with Behcet’s disease, I have to admit: it is one interesting illness. Over the 25+ years since my diagnosis, I’ve amassed quite a collection of Behcet’s info. Now I’m going to share some of the disease’s most interesting facts with you.

1. You’re more/less likely to be [insert unlikely event] than diagnosed with Behcet’s

In the United States, it’s estimated that roughly 0.38 out of every 100,000 people have Behcet’s disease.⁶ That’s about 1 out of every 260,000 people. So the odds of having Behcet’s are about 260,000:1. Not quite one in a million: More like four in a million.

The good news is, if you’re just some random American with no signs or symptoms of Behcet’s disease, it’s incredibly unlikely you have it. The bad news, of course, is for people like me who definitely do have it. Well, not so much bad news as it is just plain old bad luck.

Exactly how unlucky are we? What other rare events are more common than a Behcet’s diagnosis? How about less common? When comparing the unlikelihood of a situation to something known to be rare, two things come up more than anything else: winning the lottery and getting struck by lightening. Let’s see how Behcet’s stacks up next to them.

In any given year, the odds of being struck by lightening in the United States are about 1 in 1.2 million. But over the course of an entire lifetime (~80 years), the odds of getting struck are 1 in 15,300.⁷ That means you’re 17 times more likely to get struck by lightening at some point in your life than to be diagnosed with Behcet’s disease.

What about winning the lottery? This one’s harder to calculate because it’s impossible determine the odds of winning over the course of a lifetime, as there are many variables to consider (games played, frequency of play, etc.). However, we can compare winning any one drawing with getting diagnosed.

Take Powerball, easily one of the most popular lottery games (if not the most popular). The odds of winning the jackpot is 1 in 292 million – give or take a few hundred thousand.⁸ That means you’re more likely to have Behcet’s disease than to win the Powerball jackpot with a single ticket – a lot more likely: 1,123 times more likely, to be precise.

Here are a few more comparisons:

• The odds of dying in a motor-vehicle accident are 1 in 107.⁹ That means you’re 2,430 times more likely to be killed in a car crash than diagnosed with Behcet’s.
• The odds of dying in an earthquake are 1 in 159,890.¹⁰ That means you’re almost twice as likely to die in an earthquake than be diagnosed with Behcet’s disease.
• The odds of being killed by a hurricane are 1 in about 62,000.¹¹ That means you’re about 5 times more likely to be diagnosed with Behcet’s.
• And lastly, the odds of being diagnosed with Multiple Sclerosis (MS) in the US are about 8.3 per 100,000.¹² That means you’re about 22 times more likely to be diagnosed with MS than Behcet’s.

As you can see, Behcet’s disease is rare. It’s not as rare as winning the lottery or being killed by an earthquake, but it’s rare. So if you’ve been diagnosed, you’re either really lucky or really unlucky – depending on how you look at it.

2. Behcet’s disease doesn’t discriminate

Behcet’s disease is a type of vasculitis, a group of disorders that cause inflammation in blood vessels. There are many different types of vasculitis including Kawasaki disease, Takayasu’s arteritis, Buerger’s disease, among others.¹³

One thing all these different types of vasculitis have in common is that they either affect arteries or veins – but not both. Behcet’s disease, on the other hand, doesn’t discriminate: it can affect arteries and veins. In fact, Behcet’s is the only type of vasculitis that can affect both.¹⁴

Arteries are vessels that pump blood away from your heart and supply oxygen to your body. Then the deoxygenated blood travels through your veins, vessels that carry blood back to the heart.

The different types of vasculitis are divided into three groups based on the size of the vessels they affect: large, medium, and small-vessel vasculitis. Though Behcet’s disease is classified as a small-vessel vasculitis, it can cause inflammation in arteries and veins, small, medium, and large.¹⁵

In general, Behcet’s disease affects veins more than arteries and small vessels more than large ones.¹⁶ But inflammation in large arteries is possible. In that respect, Behcet’s doesn’t discriminate: it’s an equal opportunity inflamer.

However, Behcet’s disease can be a tiny bit sexist. Although it affects both men and women, vascular involvement is more frequently seen in men.¹⁷ That doesn’t mean women with Behcet’s don’t experience blood-vessel inflammation, too – just not as often as us guys (lucky us!).

What about other Behcet’s symptoms? Do some affect guys more than gals? Do some affect the ladies more than the fellas? Up next, we’ll explore some gender differences in Behcet’s disease.

3. Behcet’s disease does discriminate

Both men and women can be diagnosed with Behcet’s disease. And while either gender can experience any of Behcet’s symptoms, some are more common in one or the other.

As mentioned above, vascular involvement is more common in men with Behcet’s than women. And, unfortunately for us guys, so is serious ocular (eye) and neurological involvement.¹⁸

A study published in the American Journal of Ophthalmology looked at 880 patients with Behcet’s uveitis (inflammation of the uvea, part of the eye).¹⁹ After 5 years, the risk of losing useful vision in male patients was 21% and 10% in women. After 10 years, the risk was 30% for men compared to 17% for women. The researchers concluded that Behcet’s uveitis is (generally) more severe in men and more likely to result in vision loss.

Another study published in the same medical journal looked at childhood-onset Behcet’s uveitis.²⁰ The researchers found that uveitis was more common among males diagnosed with Behcet’s disease than females.

Behcet’s disease can affect the nervous system (including the brain). This is referred to as Neuro-Behcet’s. Estimates vary widely as to what percentage of people diagnosed with Behcet’s experience neurological involvement, anywhere from 2.2-50%.²¹

Though Neuro-Behcet’s can affect both men and women, it is more commonly seen in men. The men-to-women ratio for Neuro-Behcet’s is 2.9:1.²² In other words, men diagnosed with Behcet’s disease are about 3 times more likely to experience Neuro-Behcet’s than women.

When it comes to some of the other common symptoms of Behcet’s disease like oral aphthous ulcers, genital ulcers, and arthritis, men and women are affected at roughly the same rate.²³ But there’s one more way Behcet’s discriminates – mortality rate. While Behcet’s has an overall mortality rate of about 5%, men are a lot more likely to die from Behcet’s-related complications than women.²⁴

4. You’re saying it wrong

I’ll never forget the day in 1997 that I got diagnosed with Behcet’s disease. I was at the Mass Eye and Ear Infirmary in Boston being seen by one of the leading retina specialists in the country. He sat me down and said in a matter-of-fact tone, “You have Behcet’s disease,” as if all sixteen-year-old kids are supposed to know what that is.

This doctor – who’s name I won’t mention – pronounced it buh-shets disease. And the rheumatologist I began seeing soon after also pronounced it buh-shets. So, naturally, I assumed they were saying it correctly. These men were, after all, highly accomplished doctors.

Well, they were both wrong. And so have most of the doctor’s I’ve seen since. You see, Behcet’s disease was named after the Turkish dermatologist Hulusi Behçet.²⁵ You may notice that little mark under the letter c. That’s called a cedilla (sometimes spelled cedille or cedilha) and it changes the pronunciation of the letter.

Cedillas are a little hook or tail under certain letters and are used in several languages. In French, a cedilla under the letter C gives it a s- sound. However, in Turkish, a cedilla under the letter C gives it a ch- sound.²⁶

Since the written word was the most common form of communication in Dr. Hulusi Behcet’s day, a lot of people had only seen his name in writing. And a lot of those people assumed the cedilla in his last name was French, not Turkish.

Because of this, the disease he wrote extensively about became pronounced over time as buh-sets or buh-shets. But that’s not the way Dr. Hulusi Behcet’s family name was pronounced. Since it’s a Turkish name, the C-cedilla follows the Turkish rules: it’s pronounced not like s- but like ch-. So, if the name of the disease stayed true to the doctor it’s named after, it would be pronounced bay-chets disease.

5. To B or not to B – HLA-B, that is

The cause of Behcet’s disease is believed to be at least partly genetic. And no other gene has received nearly as much attention as the HLA-B51 gene (HLA stands for human leukocyte antigen).

Nearly 40 years ago in 1982, researchers published a paper that demonstrated a close association between Behcet’s disease and the HLA-B5 series of genes located on the sixth chromosome, particularly B51.²⁷ Since then, as genetic science has progressed, lots of other research has been done on HLA-B51 and other genes related to Behcet’s.

It’s estimated that between 50-80% of people diagnosed with Behcet’s disease are positive for the HLA-B51 gene.²⁸ However, just because you test positive for HLA-B51 doesn’t mean you necessarily have Behcet’s. In fact, 20% of healthy individuals (people with no signs or symptoms of Behcet’s or anything else) have the HLA-B51 gene.

Another gene associated with Behcet’s disease – though much less strongly – is HLA-B52. A study done in Israel, a country with a high prevalence of Behcet’s, found HLA-B51 present in 63% of patients with Behcet’s and HLA-B52 in 21%.²⁹ While that might not sound like a high percentage, it’s more than twice that of the control group.

Lastly, I’ll mention the HLA-B27 gene. This one is associated with several diseases including reactive arthritis, inflammatory bowel disease, ankylosing spondylitis, psoriasis, and anterior uveitis.³⁰ HLA-B27 has also been shown to be associated with Behcet’s disease, though not nearly as strongly as HLA-B51.³¹

I’m not sure about HLA-B52, but I personally have the HLA-B51 and HLA-B27 genes. I did uncover a bit of good news for those of us with both genes. At least according to one study published in 2007, patients diagnosed with Behcet’s uveitis who tested positive for both HLA-B51 and B27 had more favorable prognoses.³²

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6. What’s in a name?

“A rose by any other name would smell as sweet.” – from Romeo and Juliet by William Shakespeare³³

You may recognize that line from the famous Shakespeare play. Juliet was using metaphor to comment on Romeo’s last name. The point of her beautifully worded phrase is that names don’t matter. What something is matters, not what something’s called.

I have a 21st-century reimagining of that quote as it relates to chronic illness, particularly Behcet’s:

A disease by any other name would suck just as bad.

What most of us know as Behcet’s disease has actually gone by many different names over the years. You may have even heard some of them yourself. In modern times, aside from Behcet’s disease, it’s also commonly referred to as Behcet’s syndrome.

Even though both terms are describing the same illness, they mean different things. A syndrome is a recognizable collection of symptoms and physical findings that indicate a specific condition with no obvious, direct cause. A disease, on the other hand, is a recognizable collection of symptoms and findings where medical science has identified a cause.³⁴

Behcet’s lands somewhere in between the two. While there is evidence pointing to causal genetic and environmental factors, more research is needed before these links can be fully understood. So, technically, for now it should be called a syndrome. However, I’ve always like the name Behcet’s disease better. You may have noticed this in my writing. I tend to use disease and spell Behcet’s without the cedilla under the letter c.

But these two are hardly the only names Behcet’s has gone by over the years. A contemporary of Dr. Behcet, a Greek physician named Benediktos Adamantiades, also made contributions to the study of this illness. Because of this, sometimes it’s been referred to as Adamantiades’ syndrome or Adamantiades-Behçet syndrome.³⁵

Before 1947, Behcet’s disease went by many different names. In addition to the ones mentioned above, it sometimes went by Trisymptom Behcet, Morbus Behcet, and a couple others. Obviously, this got confusing. So in 1947 at the International Congress of Dermatology in Geneva, an official name was decided upon: Morbus Behcet.³⁶

Over time, the use of Morbus Behcet became less and less frequent. Nowadays, the illness is almost always referred to as either Behcet’s disease or syndrome. However, there is one more name that it sometimes goes by: the Silk Road disease.

The Silk Road was a trading route that spanned from Southeast Asia all the way to Southern Europe from the 2^nd century BCE until the 18^th century CE.³⁷ Behcet’s disease, for reasons that aren’t entirely understood, is much more common in countries along this ancient trading route. Because of this, Behcet’s is sometimes called the Silk Road disease.³⁸

7. Variation by location

While there are people diagnosed with Behcet’s disease all over the world, some countries have much higher rates than others. As mentioned above, countries along the old Silk Road trading route seem to have especially high rates of Behcet’s.

In the United States, Behcet’s is reported to affect between 0.12-0.33 people per 100,000.³⁹ Turkey on the other hand, the country with the highest prevalence of Behcet’s disease, affects 421 out of every 100,000 people.⁴⁰ That means if you’re Turkish, you’re up to 3,508 times more likely to be diagnosed with Behcet’s than if you’re American!

Turkey and America are on opposite ends of the prevalence spectrum with all other countries falling somewhere in between. Here are the prevalence rates for some of them (per 100,000 people) ranked highest to lowest:⁴¹

• Turkey: 20-602
• Israel: 50-185
• Iran: 16.7-100
• Saudi Arabia: 20
• Iraq: 17
• Japan: 7-13.5
• Italy: 4.1-15.9
• France: 7.1
• China: 2.62
• Portugal: 1.53
• Germany: 0.9
• United Kingdom: 0.64
• USA: 0.12-0.33

As you can see, Behcet’s is much more common in places like the Middle East than it is in most of Europe or North America. So, if you’re diagnosed with Behcet’s disease in Turkey, you’re unlucky. If you’re diagnosed in the UK or USA, you’re really unlucky.

8. Discrimination by location

You know the symptoms of Behcet’s disease can affect men and women differently (#3). You also know that different countries have different rates of the illness (#7). But did you know that in some countries Behcet’s is more common in women and in others it’s more common in men?

Not only does your location determine how likely you are to have Behcet’s disease – your gender does, too. Here in the United States, Behcet’s is almost 3 times more common in women than it is in men (0.38:1 male-to-female ratio).⁴²

That means I’m even unluckier than I’d previously thought. If the prevalence of Behcet’s disease in the United States is 0.38⁶ per 100,000 people and it’s 3 times more common in women, the odds of me (as an American male) having Behcet’s is about one in a million. What luck!

The United States isn’t the only country where Behcet’s is more common in women. Here are some others and their male-to-female ratios:⁴³

• Scotland: 0.36:1
• Spain: 0.5:1
• Korea: 0.63:1
• Israel: 0.64:1
• Brazil: 0.69:1

In some countries, Behcet’s disease is more common in men than it is in women. Kuwait has the highest male-to-female ratio at 4.9:1.⁴⁴ In other words, Kuwaiti men are about five times more likely than Kuwaiti women to have Behcet’s.

Here are some other countries where Behcet’s disease is more common in men along with their male-to-female ratios:⁴²

• Iran: 1.19:1
• France: 1.32:1
• Italy: 2.4:1
• Iraq: 3:1
• Saudi Arabia: 3.4:1
• Russia: 3.7:1

As you can see, prevalence by gender varies greatly depending on the country. In some, Behcet’s disease is much more common in men. In others, women. And in some countries like Portugal, men and women are equally affected.⁴⁵

9. Puzzle pieces

If you want to find out how well you know a subject, you can take a test. If you want to find out if you have strep throat, influenza, or a sexually-transmitted disease (STD/STI), you can take a test. But if you want to find out if you have Behcet’s disease, you have to put together a freakin’ puzzle.

Unfortunately, there is no single test you can take that will tell you if you have Behcet’s or not. Doctors have to make the diagnosis based on your symptoms, family history, and other clues. In other words, they have to first collect the puzzle pieces, then put them together.

In 1990, the International Study Group (ISG) for Behcet’s disease put together a set of evidence-based diagnostic criteria for the first time.⁴ According to the criteria, a diagnosis of Behcet’s can be made when a patient has recurrent oral ulceration with at least two of the following: eye lesions, skin lesions, recurrent genital ulceration, and/or a positive pathergy test.

While the diagnostic criteria of the ISG was a step in the right direction and helped to advance Behcet’s disease research, it had some flaws. So in 2006, the International Criteria for Behcet’s Disease (ICBD) was created to replace the ISG criteria.⁴⁶ In addition to the five items listed in the ISG criteria, two more were added to the ICBD: vascular and neurological manifestations.

The ICBD uses a point system to make the diagnosis of Behcet’s disease. Each symptom is worth either 1 or 2 points. After adding up the points, a score of 4 or higher indicates a Behcet’s diagnosis. Here are the criteria and how many points they’re worth:⁴⁷

• Ocular lesions: 2
• Genital aphthosis: 2
• Oral aphthosis: 2
• Skin lesions: 1
• Neurological manifestations: 1
• Vascular manifestations: 1
• Positive pathergy test: 1

You may be wondering what this positive pathergy test criteria is all about. Didn’t you say there were no tests for Behcet’s? While there is no single test that can tell you whether or not you have Behcet’s, there are some tests that help make the diagnosis. A positive pathergy test is one of them.

The way this test works is simple. A doctor will cause a tiny amount of trauma to your skin (usually by pricking it with a needle) and see how it reacts over the next 48 hours. People with Behcet’s disease often – but not always – develop a papule or pustule at the site of trauma. This is considered a positive pathergy test and it’ll earn you a point on the ICBD.⁴⁸

10. There’s never been a better time to have Behcet’s disease

I was diagnosed with Behcet’s in 1997. Back then, things were a lot different. It was almost impossible to find information about Behcet’s disease. At the time, only one or two books had been published on the subject. And while the internet did technically exist in the nineties, it wasn’t like it is today. You couldn’t just Google Behcet’s disease, pop into a Behcet’s Facebook group, or use one of the other online Behcet’s disease resources that are readily available today.

Treatment has come a long way over the past 25-or-so years, too. Entirely new classes of drugs have come on the market, like biologics. These include drugs like infliximab (Remicade) and adalimumab (Humira) and have been highly effective at treating the symptoms of Behcet’s for many patients.⁴⁹

Of course, being diagnosed with Behcet’s disease at any time in history sucks. But if you’ve recently been diagnosed, the illness will be much easier to manage now than it would’ve been just a couple decades ago. I can’t even imagine having Behcet’s a century ago. That must’ve really sucked.

In the years following my diagnosis, I looked everywhere for information about Behcet’s. As I mentioned, there were only a couple books out at the time. But now there are several excellent books about Behcet’s disease. Some of them are medical/reference books packed with information for healthcare providers and curious patients. Others are biographies and memoirs that tell emotional tales about what it’s like living with this rare illness.

The first two books about Behcet’s I was able to get my hands on were both written by Joanne Zeis. One of them, a book titled You Are Not Alone: 15 People With Behcet’s, came out just a month before I was diagnosed. Whether I actually was or not, I sure as hell felt alone. But that book made me realize others were out there with the same lousy illness. I’d highly recommend it and the other books Joanne has written about Behcet’s.

In 2016, a woman named Vicki Hamm published a book about her son’s battle with Behcet’s disease. Ty’s Story: A True Story about Living with Behcet’s Disease and the Afterlife is a beautifully written, heart-wrenching tale overflowing with tragedy, love, and spirituality.

Last and quite possibly least, if you’re interested in reading all the dirty details of my experience living with this awful illness, I wrote a memoir about it that was published at the end of last year. It’s called Finding Happiness Through Pain and Embarrassment: My Life With Behcet’s Disease – A Memoir. I tell the story of how, in spite of all the pain and suffering Behcet’s has caused me, I’ve still managed to stay positive and find happiness in the unlikeliest of places.

Conclusion

And there you have it: 10 interesting facts about Behcet’s disease. As much as it sucks living with Behcet’s, I have to admit, it is an interesting illness. I’ve learned a lot about it over the years and look forward to learning even more in the future.

What do you think of these ten interesting Behcet’s disease facts? Did you already know some of them? All of them? Think I missed something? Please leave your answers in the comments section at the bottom. And if you haven’t already, please sign up for the EM newsletter below.

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