She has seen a number of specialists, but this is tiring for her and usually involves travel. We live in a small Canadian province without much diversity, so it was only by chance that the ophthalmologist she saw recognized the symptoms because he was from another country.

She has been getting injections in her eye and having both eyes checked frequently. Her throat is getting worse. Should she stop eating and drinking and have IVs until her throat heals? When she does see a physician she hasn’t seen before, they question whether she has lupus, Lyme or Crohn’s disease. She’s very discouraged.

Could you tell us what you think the treatment should be and if there are any centers in Canada or the United States that have expertise dealing with this?

DEAR READER: I must agree that your sister was extremely lucky to have had an ophthalmologist who recognized her symptoms and could put a name to her condition. My guess is he may be Asian, since Behcet’s is most common in the Far East and Mediterranean areas. The disease is rare in the United States and Canada, but cases do surface. It is named after a Turkish dermatologist, Hulusi Behcet, who documented aphthous oral ulcers, recurrent eye inflammation and genital lesions in the 1930s. Epidemiology of the disease is not well understood.

The syndrome is an idiopathic disorder classified as a form of vasculitis (blood-vessel inflammation). Because of the diversity of the blood vessels affected, it can occur throughout the body but is most common in the eyes, mouth, lungs, joints, genitals, brain, GI tract and on the skin. When the eyes are affected, inflammation can appear in the front or back, or sometimes in both areas at the same time. When the mouth, lips, tongue and inside of the cheeks are affected, the lesions are often painful, large and numerous. Aneurysms in the lungs can rupture and lead to massive hemorrhaging. Painful ulcers can develop on the vulva or scrotum. Involvement of the central nervous system is a dangerous manifestation. When the disease involves the white matter of the brain and brainstem, stroke, headache, confusion and other conditions can develop. The GI tract can be involved from the mouth to the anus. Inflammatory bowel disorders such as Crohn’s disease share similar side effects that may be difficult to differentiate from Behcet’s.

Diagnosis of Behcet’s can be extremely difficult to clarify. More than anything, symptoms such as oral or genital ulcers and evidence of vasculitis through biopsy of an involved organ are what lead a physician to make the call. Diagnostic guidelines involve two of the following: eye lesions and/or skin lesions a minimum of three times a year; recurrent genital ulcerations; and/or pathergy testing, which is a simple needle prick on the arm. A small red pustule occurring at the site of the needle insertion within two days is classified as positive. Pathergy is not proof positive of the disease, because many people from the Mediterranean will test positive and may not have Behcet’s.

For lesions confined to the mouth, genitals and skin, topical steroids and some oral drugs may be effective. When symptoms become particularly troublesome, corticosteroids may be beneficial. Some people may be placed on low-dose prednisone for control. When eye or central-nervous-system involvement occurs, high doses of prednisone and another form of immunosuppressive treatment are often necessary for control. Thalidomide, a drug banned in the United States because of a connection with birth defects, has been found to be effective in severe mucocutaneous cases of Behcet’s, even though there is a connection with the development of peripheral neuropathy in patients who take it for extended periods of time.

I do not believe that fasting is an answer for your sister, even with her troublesome throat lesion. In terms of finding a physician to provide care, while the condition is uncommon in the United States and Canada, it has been studied and documented extensively. In fact, a 1998 article by David Hellmann, published and copyrighted by the American College of Physicians in the Medical Knowledge Self-Assessment Program, edition 11, Rheumatology Section, covered Behcet’s. Furthermore, your sister could check with the Johns Hopkins Vasculitis Center to determine whether any trials she can participate in are under way. Good luck.

My niece supposedly had a stroke. She then had X-rays that showed she had a small spot of bleeding in her brain. She went to several doctors because she had all kinds of things happening to her like headaches that nothing helped that disappeared, only to be replaced by seizures. She was having trouble walking and her memory was getting worse. Her legs would give out and she would fall. One arm became limp and a host of other things.

All this lasted for two years and was accompanied by numerous hospitalizations. Finally her doctor told her that both carotid arteries were plugged. He said it was caused by plaque. She kept getting worse so her husband changed her doctor and switched hospitals.

The new physician, after seeing her, referred to a teaching hospital. She had a couple of tests and more X-rays. She was told she didn’t have plaque buildup in her carotid arteries, but rather infection, and was then diagnosed with vasculitis.

I am told it is a very rare blood infection. She is now taking large doses of prednisone and her physician says she will likely get most of her memory back. She is getting better already and can now walk by herself again (but still has someone with her at all times, just in case). She has also regained use of her arm.

My reason for writing is that I don’t understand why her new doctor hasn’t let the whole world know about this new disease he has found. He said he has been a doctor for more than 30 years but has only ever seen one other case of vasculitis. When I talked to my personal physician about it, he said he had never even heard of it.

DEAR READER:
Vasculitis is inflammation (not infection) of the blood vessels, capillaries, veins and/or arteries. When this occurs it can weaken and narrow the vessels which may result in blockage. With decreased blood flow, less oxygen can be supplied to muscles and other tissues. If the inflammation is not brought under control, tissue, muscle and organ damage may occur. If the damage is severe, it may result in death.

There are several disorders that fall into the vasculitis family and include Churg-Strauss Syndrome, Kawasaki disease, Wegener’s granulomatosis, polymyalgia rheumatica and more.

While each disorder is different, many of the symptoms overlap. The primary distinguishing point is the type of blood vessels that are affected (small, medium or large). General symptoms include shortness of breath/cough, joint pain, asthma, fever, rash, fatigue/weakness, abdominal pain, visual changes and more.

Vasculitis can affect anyone at anytime but certain forms affect specific age groups more than others. It is considered to be an autoimmune disorder, meaning that the body attacks itself.

There is no known cure, but with early diagnosis and treatment, most sufferers can lead relatively normal lives. Treatment can include steroids (prednisone and others), immunosuppressive medications and antibiotics.

I suggest you and your daughter return to her physician to discuss her condition. Vasculitis by itself is not a diagnosis, it is simply a broad term to identify a primary symptom. If you are one of her care-givers, it is also vital that you understand what the condition is, what type of treatment she is on and what warning signs to look for if she has a relapse.

To give you related information, I am sending you a copy of my Health Report “Stroke”. Other readers who would like a copy should send a self-addressed, stamped number 10 envelope and $2 to Newsletter, PO Box 167, Wickliffe, OH 44092. Be sure to mention the title.