by Joanne Zeis, c2004; do not copy/distribute without permission.
Behcet's Disease Books and Resources
Email: Joanne Zeis
Before I start any explanation of Behcet's mortality [death] and morbidity [health complications] statistics, here's my disclaimer: I'm not medically trained. Information contained in this section is based on medical research study results as of August 2004. Any questions about material in this section should be directed to your health care provider(s), who are the only people authorized to give medical advice and treatment for your care!
Statistics and published medical research studies are useful -- they can give a snapshot of what has happened to a specific group of people over a specific period of time. The reports can help doctors make treatment decisions, and help patients live more comfortable lives. The limitation to these statistics? YOU are not a statistic! You are a living, breathing person with a life, family and friends. It's easy to look at statistics and get worried or frightened about complications that might happen in the future, but the reality is that people "beat the odds" all the time, whether they have cancer, Behcet's, or some other illness. Having BD is no more (and probably much less) of a death sentence than scores of other, more well-known diseases. As a 25-year veteran of Behcet's, I believe in the power of positive thinking... it can't hurt, and could help your situation. Spiritual beliefs? If you have them, lean on them! Research studies have shown positive health results through religious participation and prayer, among other spiritual activities.
With that said, use the following information to your best advantage. Educating yourself (NOT scaring yourself!) is the primary goal here. If you have the right information, you can then know what potential complications to watch out for. In many cases, prompt treatment can minimize possible long-term health problems.
Please feel free to share the information on this web page with your doctor(s), so they're aware of any concerns that you might have.
Above all, stay positive! and be well.
All the best,
Behcet's Research Study Statistics on Mortality/Morbidity
Here is the summary of the most recently-released study on health complications and deaths related to Behcet's. It's very significant because it's the only study to follow a specific group of Behcet's patients for 20 years. While this study's preliminary results were printed in the "Essential Guide to Behcet's" in 2003, the final results of this study were published in a medical journal after the "Essential Guide" went to press. (The final mortality rate in the recent medical journal article was calculated to be 9.8%, rather than the 6% quoted in the EG). It's important to note that this study took place in Turkey, and all results may not apply to patients in other countries such as the U.S. or U.K. (traditionally considered to have less serious BD-related symptoms than Middle East and Far East countries that are the "hotbed" of Behcet's).
Here is the summary:
Medicine (Baltimore). 2003 Jan;82(1):60-76.
The long-term mortality and morbidity of Behcet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center.
Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, Yurdakul S, Yazici H.
Behcet Syndrome Research Center, Cerrahpasa Medical Faculty, University of Istanbul, Turkey.
We surveyed the 20-year outcome of a cohort of patients with Behcet syndrome with emphasis on both mortality and morbidity. During 1999 and 2000, we collected outcome information on 387/428 (90.4%) of a cohort of patients (262 male, 125 female) who had registered in a dedicated outpatient clinic between July 1977 and December 1983. In 245/345 (71.0%) patients, outcome information was based on a formal hospital reevaluation, and in the remaining patients, on detailed telephone interviews. Forty-two patients (9.8%) (39 male, 3 female) had died, mainly due to major vessel disease and neurologic involvement. Mortality, as measured by standardized mortality ratios (SMR), was specifically increased among young males, among whom morbidity was also the highest. However, the SMR tended to decrease significantly with the passage of time. The same was also true for all mucocutaneous and articular manifestations. Both the onset of eye disease and its greatest damage were also usually within the first few years of disease onset. These suggest that the "disease burden" of Behcet syndrome is usually confined to the early years of its course, and in many patients the syndrome "burns out." However, central nervous system involvement and major vessel disease are exceptions. They can have their onset late (5-10 yr) during the disease course. As reflected in the mortality figures, the disease was less severe among the females for almost each disease manifestation. There were no female patients with arterial aneurysms. Severely impaired vision did not always mean an eventual loss of useful vision, and those patients with a late onset of eye disease had a better visual prognosis.
The following section gives a general overview of the more serious complications of Behcet's. It's from "Essential Guide to Behcet's Disease" (c2003 by J. Zeis):
Am I going to die from Behçet's Disease?
The majority of BD patients have a normal lifespan. According to Orloff (1999), the mortality rate of patients with Behçet's disease is 3-4%(17), although Kural et al's study in 2000 suggests a higher 6% rate (26 of their 428 Behçet's patients who were followed for twenty years died of complications directly relating to BD).(164) [JZ: final published statistics raised the 20-year mortality rate to 9.8%]. Put into perspective, if 14,000 people in the US have Behçet's disease right now, anywhere from 420 to 840 of those patients may eventually die of complications from BD. The remaining 13,000+ patients will die of other causes - including old age. However, it does appear that young male patients may need to be followed more closely. Yazici et al (1996) looked at 152 male and female BD patients over a 10-year period, and found that the mortality rate among 15- to 24-year-old male patients, in particular, was higher than one would expect to see in the general population; it was also higher than any other patient age group.(18) Kural's study came to the same sobering conclusion about young male patients.(164) Yazici did note that all of the patients who died (a total of six men out of 152 men and women in the original study) tended to have more severe disease symptoms at the time they entered the study. The specific causes of death were: pulmonary arterial aneurysm, inferior and superior vena cava syndromes, upper GI hemorrhage, CNS involvement, and cerebrovascular accident. Pulmonary artery aneurysms, vena caval thrombosis, CNS complications, amyloid nephropathy, and one suicide were the causes of death among the patients in Kural's study.(164) In another study by Park et al (1993), the causes of death in seven Behçet's patients (six men and one woman) were: GI bleeding, bowel perforation, superior and inferior vena cava syndrome, aortic regurgitation, cerebrovascular disease, and lung abscess.(19) In a 1999 study by Kidd et al, two out of 35 neuro-Behçet's patients, followed for a median of three years, died of aspiration pneumonia with severe brainstem impairment.(112) The most recent study (Al-Saleh et al, 2000) followed 208 men and women over a twelve-year period in Saudi Arabia. During that time, five men and one woman died (2.9% of the total enrolled patients). The causes of death were: Budd-Chiari syndrome in three cases; pulmonary hemorrhage (one case); superior and inferior vena cava obstruction and sepsis (one case); and cerebrovascular accident in the remaining case.(69)
As the above studies suggest, women do not appear to die from Behçet's complications as frequently as men. In spite of Walsh and Rau's September 2000 study showing that autoimmune disease is a leading cause of death among young and middle-aged women(20), Behçet's disease does not specifically appear on their accepted list of 24 autoimmune diseases followed for the study. The most frequent causes of autoimmune mortality for women are: rheumatic fever and heart disease, rheumatoid arthritis, multiple sclerosis, systemic lupus erythematosis, and scleroderma. However, glomerulonephritis [a serious form of kidney inflammation] also appears as one of the top six diseases on Walsh and Rau's list, and glomerulonephritis is known to be a complication of Behçet's disease(21).
In 2000, Dilsen suggested that the ultimate outcome of any case of Behçet's is dependent on whether or not major (vital) organs have been affected. For the purposes of his study, major organs were defined as: eyes, CNS [central nervous system], deep veins, arteries, heart, bowels, lungs, and the presence of amyloidosis [the creation and deposit of amyloid - a starch-like substance - in tissues and organs, especially the liver, spleen and kidneys during some chronic diseases]. He found that vital organ involvement occurred more frequently in patients with the following characteristics: male; age less than 40; disease starting before the age of 25, first symptom is not aphthous ulceration; late diagnosis, and lack of treatment.(99)
Neuro-Behçet's (involvement of the central nervous system) has a worse prognosis than many other forms of Behçet's, justifying its inclusion in Dilsen's "poor outcome" list. In 1999, Akman-Demir et al estimated a mortality rate of approximately 11% during a 5-year follow-up period of 200 neuro-BD patients. However, the researchers were able to list certain factors that influenced the ultimate outcome. For example, if a patient had normal CSF (cerebrospinal fluid) results during an acute neuro-BD attack, there was less resulting disability and a more stable disease course in the long term. However, a high cellular and/or protein count, or brainstem-type involvement, usually resulted in a worse prognosis.(138)
According to Wechsler et al (1999), cardiac manifestations [heart problems] can also create serious complications, and a higher-than expected mortality rate. Possible cardiac problems include coronary artery disease, pericarditis [inflammation of the membrane surrounding the heart], myocardiopathy [disease of the middle layer of the walls of the heart], and valve disease, among other difficulties. Wechsler noted that BD-related coronary artery disease can occur even in young patients, first seen as angina or a heart attack; it has a significant 20% mortality rate in the months or years following diagnosis.(145)
Two recent studies advise caution when performing surgery on Behçet's patients for cardiovascular symptoms. In a study by Kwak et al (2000), fifteen cardiac operations were performed on ten patients over six years. Five of the ten patients died of cardiovascular complications following their surgeries, including three who died of bleeding from a false aneurysm at the surgical site. Surgeries included: aortic valve replacements, Ross procedure, cusp replacement of the aortic valve, and Bentall's procedure.(118) Lee et al (2000) followed nine patients who underwent a total of 17 aortic valve replacements. On average, 44% of the patients died within nine months of the surgery. Deaths were caused by heart failure or infection associated with detachment of the prosthetic valve, or because of leakage around the heart valve(s).(119) Authors of these studies strongly urge either the use of immunosuppressive drugs following surgery, or the pre-surgical use of steroids and other medications, to reduce potentially fatal complications.
While amyloidosis [described earlier] occurs in less than 2% of Behçet's patients, it carries a high 50% mortality rate over 1-11 years. Melikoglu (2000) found that peripheral and pulmonary arterial involvement, and arthritis(106) tend to occur more frequently in patients who eventually develop amyloidosis.
And finally, the mortality rate of BD patients who develop [the rare complication of] acute Budd-Chiari syndrome is 61%.(17) Budd-Chiari syndrome occurs when major veins of the liver are blocked. Orloff's 1999 study states that "Because the prognosis for long survival is quite good in Behçet's disease, early diagnosis of acute Budd-Chiari syndrome is imperative, and prompt treatment by portal decompression [side-to-side portacaval shunt] is indicated."(17)
c.2004 by Joanne Zeis
Do not copy/distribute without permission.
Last revised: 8/14/04