How is Behcet'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 Behcet's Disease
(est. in 1989), these are the primary symptoms of Behcet'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]
- recurrent genital ulcerations [resembling oral ulcers, usually painful, 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 Behcet's symptoms. However, North American patients rarely test positive, even during disease activity.]
Because of a lack of laboratory tests specific for Behcet's, however, 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 [problems with kidneys]
- 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 may 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.
- Morelli S, Perrone C, Ferrante L, Sgreccia A, Priori
R, Voci P, et al. Cardiac involvement in Behçet's
Patients with Incomplete BD may or may not progress to a full-blown case of Behçet's.
More basic information about Behcet's disease:
What is Behcet's disease?
How is Behcet's diagnosed? (including International Classification Criteria of 1989)
Other symptoms related to Behcet's
What causes Behcet's, and how will it affect my family and my life?
What is it like after 30 years of symptoms?
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