Also known as Behcet’s Syndrome, Behcet’s Disease is a rare disorder which causes inflammation of blood vessels throughout the body. This disease can eventually lead to numerous other signs & symptoms which may seem unrelated to this condition in the beginning. These can include lesions, skin rashes, eye inflammation, mouth sores & genital sores. Moreover, effects of Behcet’s disease vary from one person to another & may also in cases clear up on its own. Treatment of this condition involves medications so as to reduce signs & symptoms of the disease & to prevent other serious complications including blindness.
Signs & Symptoms of Behcet’s Disease
Behcet’s disease signs & symptoms vary from one person to another. Quite often it may disappear & recur on its own. Symptoms may also become less severe over time in some cases. Moreover, these signs & symptoms generally depend upon which part of the body is affected. Areas which are commonly affected by Behcet’s disease include the following.
- Mouth – These are generally painful mouth sores which look quite similar to cancer sores. These also are the most common sign & symptom of Behcet’s disease. Initially they start as round & raised lesions in mouth which quickly turn into painful ulcers. Although they often recur, these sores usually heal within 1 – 3 weeks of time.
- Genitals – Behcet’s disease patients may also develop sore on genitals. These are red & open sores which commonly occur on scrotum for males or on vulva among females. These sores are usually painful & may also leave scars.
- Skin – Skin problems from Behcet’s disease can vary. Some people may also develop acne-like sore on the body, while others may develop raised red & tender nodules on skin, especially upon lower legs.
- Eyes – Behcet’s disease may also cause inflammation in the eye in some people. This condition is known as uveitis & may usually cause pain, redness & blurred vision either in one or both eyes. Moreover, in Behcet’s disease patients this condition may often come & go.
- Vascular System – Appearing in both veins & arteries, this inflammation in blood vessels sign & symptom of Behcet’s disease causes pain, redness & swelling in arms or legs & will usually result in a blood clot. Moreover, inflammation within large arteries can lead to some complications like narrowing or blockage of the blood vessel & aneurysms.
- Joints – Swelling of joints & pain may often affect knees among people with Behcets disease. Wrists, elbows & ankles may also be involved in some cases. Signs & symptoms usually last between 1 – 3 weeks & generally go away on their own.
- Brain – Behcet’s disease is also found to cause inflammation within brain & the nervous system which can lead to headache, disorientation, poor balance, fever & stroke.
- Digestive System – Behcet’s disease is also found to cause a variety of signs & symptoms which affect the digestive system of patients. These include bleeding, diarrhea & abdominal pain.
Causes of Behcet’s Disease
No one exactly knows as to what causes Behcet’s disease. It is generally considered as an autoimmune disorder, which means that the patient’s own immune system is attacking some of his/her own healthy cells. It is also likely that environmental & genetic factors are also playing a role in development of this condition. Several genes have also been found to be associated with this disease. However, some researchers also believe that a bacterium or virus may be triggering Behcet’s disease among people who are having certain genes which make them susceptible to this disease.
Risk Factors Associated with Behcet’s Disease
Factors which generally increase a person’s risk of developing Behcet’s disease include the following.
- Age – Behcet’s disease is commonly found to affect both men & women in 20 – 30 years age group; although children & older adults can also develop this condition.
- Area Where Patient’s Dwell – People living in Far East & Middle East including countries like Japan, China, Iran & Turkey are more likely to develop Behcet’s disease.
- Sex – Although Behcet’s disease is found to occur in both men & women, it is usually much more severe among men.
- Genes – Having certain genes is usually associated with a higher chance of developing Behcet’s disease.
Complications Associated with Behcet’s Disease
Complications of Behcet’s disease generally depend upon the signs & symptoms of the patient. Like for example, uveitis which is left untreated will lead to decreased vision & even blindness in some people. People with signs & symptoms of eye in Behcet’s disease will need to regularly see an ophthalmologist (eye doctor) because timely treatment can help prevent this complication.
Diagnosing Behcet’s Disease
No tests can definitely determine whether a person is having Behcet’s disease. Instead doctors primarily rely upon signs & symptoms in order to diagnose Behcet’s disease. However, suspected patients can conduct blood test or other laboratory analysis in order to rule out other conditions. However, certain criteria have been established for diagnosing Behcet’s disease, but these are not always essential while diagnosing this disease. Doctors often use other factors for diagnosing this condition. Classification of criteria which is required for diagnosing Behcet’s disease includes the following.
- Mouth Sores – Because nearly anyone with Behcet’s disease will have mouth sores at some point of time, this sign & symptom is normally necessary for diagnosis. Diagnostic criteria for Behcet’s disease require mouth sores which have recurred at least 3 times during a 12 month period.
Additionally, in order to confirm diagnosis for Behcet’s disease, patients must also have at least 2 more of signs & symptoms mentioned below.
- Genital Sores – Recurrence of genital sores may indicate that the patient is having Behcet’s disease.
- Eye Problems – Ophthalmologists can conveniently identify signs of inflammation in the eyes of Behcet’ disease patients.
- Skin Sores – These include a variety of acne-like sores or rashes which may be caused by Behcet’s disease.
- Positive Pathergy Test – During a pathergy test, doctors will insert a sterile needle into the patient’s skin & subsequently examine the area a day or two later. In case pathergy test is positive, a small red bump would form under the patient’s skin where the needle was inserted. This would indicate that the patient’s immune system is overreacting to a minute injury incurred by the needle.
Initial Appointment with Doctors for Behcet’s Disease
Whenever anyone notices any unusual signs & symptoms indicating Behcet’s disease, it is time to make an appointment with the doctor. For patients who have already been diagnosed by Behcet’s disease, they must also see the doctor in case they notice any new signs & symptoms.
- Preparing for Initial Appointment
People would most often start by seeing his/her primary healthcare physician when they notice signs & symptoms of Behcet’s disease. They may subsequently be referred to a doctor who is treating arthritis or to a rheumatologist who is treating other rheumatic illnesses. These specialists would generally confirm diagnosis of Behcet’s disease. In case a rheumatologist was not involved in diagnosing Behcet’s disease, patients may however be referred to rheumatologists for managing this condition. Many patients may also be needed to see an ophthalmologist (eye doctor) for eye problems, a urologist or gynecologist for genital sores, a gastroenterologist for difficulties in digestion, a dermatologist to address skin problems & a neurologist for symptoms involving the brain and/or the central nervous system. Moreover, since these appointments with specialist doctors can often be brief & there is plenty of ground to cover, it is a sensible idea that patients be prepared. Following is some information & which is meant to help the patient get ready for initial appointment for Behcet’s disease.
- What Patient’s can do in Preparation for Behcet’s Disease Initial appointment
- Make a List of Symptoms Patients are Experiencing – This list should include everything including symptoms which may seem unrelated to the reason for which this appointment is scheduled.
- List Key Personal Information – This list should enclose all personal information including major stresses & recent changes in life.
- Make a List of All Medications – this should include medicines, vitamins & supplements including dosage.
- Ask a Friend or Family Member to Come Along – Apart from offering support, Behcet’s disease patient’s accomplice can write down information from doctors and/or other clinic staff during the initial appointments.
- List of Questions to Ask Doctors – Preparing a list of questions which can help patients make the most of the time they are having during the initial appointment. Some basic questions which patients can ask doctors include the following.
- What is the most possible cause of my symptoms?
- What are the other possible causes of my symptoms?
- Which tests will I need? Will I need to specially prepare for these tests?
- Is my medical condition temporary or long-lasting?
- What treatment options do I have?
- Which treatment option you think is best for me?
- Since I am having other medical conditions, how can I best manage these together?
- Are you having any brochures or other printed material providing information about Behcet’s disease?
- Which websites would you recommend I should refer to for more information on Behcet’s disease?
- What Doctors Generally Ask Behcet’s Disease Patients
Doctors are most likely to ask a number of questions to Behcet’s disease patients like the ones listed below.
- What types of signs & symptoms have you been experiencing & when did they begin?
- Are you having these symptoms all the time or they usually come & go?
- How severe are these symptoms?
- When did you first experience these symptoms?
- What is it, if anything, which seems to improve upon your symptoms?
- What is it, if anything, which seems to worsen these symptoms?
- Has anyone within your family ever have has a similar illness?
Treatments for Behcet’s Disease
Presently, there is no existing cure for Behcet’s disease. Doctors usually offer medications in order to control temporary flares of pain & inflammation to people suffering from mild forms of this disease. Doctors may recommend additional medications to control signs & symptoms of Behcet’s disease in all parts of the body when symptoms are more severe, along with medications which were prescribed for temporary flares.
- Treatments for Individual Signs & Symptoms of Behcet’s Disease – Doctors would usually try to control individual signs & symptoms people experience during flares with medications like the ones mentioned below.
- Skin Creams, Gels & Ointments – These include topical corticosteroid medications which are directly applied to skin & genital sores so as to reduce pain & inflammation.
- Mouth Rinses – Generally include special mouth washes containing corticosteroids & other agents which are meant to reduce pain of mouth sores & which may ease discomfort among Behcet’s disease patients.
- Eye Drops – These eye drops are also containing corticosteroids or anti-inflammatory medications which can relieve redness & pain in eyes in case inflammation is mild.
- Systemic Treatments for Behcet’s Disease – When topical medications fail to yield results, doctors generally recommend a drug known as colchicine (Colcrys). Many signs & symptoms of arthritis are found to improve with colchicines. Most severe cases of Behcet’s disease also require treatments which can control damage from this disease between flares as well. Patients having moderate to severe Behcet’s disease are generally prescribed the following medications.
- Corticosteroids to Control Inflammation – Corticosteroids like prdnisone will effectively reduce inflammation which is caused by Behcet’s disease. However, signs & symptoms of Behcet’s disease generally tend to recur among patients when corticosteroids were used alone. For this reason doctors also prescribe another immunosuppressive medication to suppress activity of the immune system along with corticosteroids. Common side effects of corticosteroids generally include weight gain, high blood pressure, persistent heart burn & osteoporosis (bone thinning).
- Medications to Suppress Immune System – Immunosuppressant drugs are meant to reduce inflammation by stopping the immune system from attacking healthy tissues within the body. Immunosuppressive drugs which play a leading role in controlling Behcet’s disease include cyclophosphamide (Cytoxan), cyclosporine (Sandimmune, Neoral, Gengraf) & azathioprine (Azasan, Imuran). Since these medications suppress actions of the immune system, they would also be increasing risk of infection. Other possible side effects of immunosuppressive drugs include kidney & liver problems, high blood pressure & low blood counts.
- Medications to Alter Immune System Response – Interferon alpha-2b (Intron A) is a medication which regulates activity of the patient’s immune system so as to control inflammation. This may either be used alone or in combination with other drugs in order to help control joint pain, skin sores & eye inflammation among people with Behcet’s disease. Side effects of interferon alpha-2b include flu-like signs & symptoms like fatigue & muscle pain. Medications which block a substance known as Tumor Necrosis Factor or TNF are effective in treating some signs & symptoms of Behcet’s disease, especially for patients who are having resistant or more severe symptoms. Good examples of TNF include etanercept (Enbrel) & infliximab (Remicade). Side effects of TNF include skin rashes, headache & increased risk of developing upper respiratory infections.
Coping & Support for Behcet’s Disease Patients
Unpredictability of Behcet’s disease makes treatment quite difficult & frustrating. However, taking good care will help patients cope better with this arthritic condition. Patient’s approach towards caring for himself/herself will depend upon the type of signs & symptoms they are experiencing on that particular day. Nevertheless, they must generally try to take the following steps.
- Rest During Flares – Whenever Behcet’s disease signs & symptoms appear, patients must take time for themselves. They should be flexible & adjust their schedule wherever possible, so that they can rest whenever required. Moreover, it is also a wise idea to keep stress at a minimum, as stress can generally aggravate symptoms.
- Be Active When Feeling Healthy – Behcet’s disease patients must indulge in moderate exercises like swimming or walking. This will make them feel much better in-between Behcet’s disease flares. Moreover, exercise will strengthen their bodies & also help keep their joints flexible alongside improving mood.
- Connecting with Others Having Behcet’s Disease – Most often, it may be quite difficult to find another Behcet’s disease patient nearby since this is a rare disorder. Patients should therefore ask their doctors about the presence of such support groups within their area. In case it is not possible to find some with this disorder close by, Behcet’s disease patients can connect online with patients who are also having this disorder.
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