How is Behçet’s diagnosed?
There is no test for Behçet’s at the moment. It is diagnosed by specific patterns of symptoms and repeated outbreaks of them.
Any other causes for these symptoms have to be ruled out first. The symptoms do not have to occur together, but can have happened at any time.
There are levels of certainty for diagnosis (and additional knowledge since the original criterion):
- International Study Group diagnostic guidelines (very strict for research purposes) (1990)
- Practical clinical diagnosis (generally agreed pattern but not so strict)
- Suspected or possible diagnosis (incomplete pattern of symptoms)
- The International Criteria for Behçet’s Disease (ICBD) (2013)
- NHS Choices Diagnosis – Diagnosing Behçet’s Disease (as at June 2018)
1. International Study Group strict research level guidelines for diagnosis
- mouth ulcers (any shape, size or number at least 3 times in any 12 months)
Along with 2 out of the next 4 ‘hallmark’ symptoms:
- genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)
- skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)
- eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
- pathergy reaction (papule >2 mm diameter, 24-48 hrs or more after needle-prick)
2. Practical clinical guidelines for patients not included in research cohorts
- mouth ulcers
Along with 1 out of the 4 ‘hallmark’ symptoms above
Along with 2 of the following symptoms:
- nervous system symptoms
- stomach and/or bowel inflammation
- deep vein thrombosis
- superficial thrombophlebitis
- cardiovascular problems
- inflammatory problems in chest and lungs
- problems with hearing and/or balance
- extreme exhaustion
- changes of personality, psychoses
- any other member of the family with a diagnosis of Behçet’s
3. ‘Suspected’ or ‘possible’ diagnosis
Usually given when someone does not have mouth ulcers or has mouth ulcers but does not have 1 of the 4 ‘hallmark’ symptoms but has other symptoms and signs of inflammation and other causes for these have been ruled out.
Symptom proportions for members of the Behçet’s Syndrome Society in the UK (survey dated 1994)
|Symptom||% of people with symptom|
|Tissue reactivity (pathergy)||
|Inflammatory bowel disease||
|Deep vein thrombosis||22|
(Other symptoms were not measured)
4. The International Criteria for Behcet’s Disease (ICBD) (2013)
International Criteria for Behcet’s Disease – point score system: scoring >4 indicates Behcet’s diagnosis
*Pathergy test is optional and the primary scoring system does not include pathergy testing. However, where pathergy testing is conducted one extra point may be assigned for a positive result.
Distribution of scores in cases and controls in both validation and training data sets along with a proposed plausibility scale of BD diagnosis is presented in Table 6.
- What is Behçet’s? Behçet’s syndrome (now more commonly referred to as Behçet’s disease) is a chronic condition resulting from disturbances in the body’s immune system.
- Similar Disorders There are several disorders that have patterns of symptoms that overlap with those of Behçet’s. In the absence of a test to confirm Behçet’s, this can make diagnosis quite difficult.
- BSS Factsheets All the factsheets listed are available free of charge from the Society, and many can be downloaded here.
- Medical Review Article
- Headache in Behçet’s Headache is one of the most continual and troublesome symptoms in Behçet’s. Some patients are lucky and hardly ever get headaches; others have daily chronic severe throbbing headaches that are very disabling. See factsheet no. 15 for further information about headache in Behçet’s.