Behcet’s Disease Syndrome Criteria

The International Study Group for Behcet’s Disease was created in 1990 with the aim to bring together the existent sets of diagnostic criteria (around 5 at the time) and create and test a comprehensive model.

Confirming a diagnosis of Behcet’s (vascular and tissue inflammation) is notoriously difficult because of the wide variability of the symptoms, which are often shared with other diseases.

Diagnostic criteria

There are 5 items (one major and four minor), from which two refer to mucous membrane manifestations, one refers to skin, another to ocular manifestations and the last one to the presence of pathergy phenomenon by pathergy test.

The major criterion is: Recurrent oral ulcerations - minor aphthous, major aphtous or herpetiform ulceration observed by physician or patient, which recurred at least 3 times in one 12-month period.

Minor aphthous is defined as painful sores, pale yellow in color, that are surrounded by swelling and redness. Their pattern of occurrence is one at a time, without any scarring left and their duration is of 1 to 2 weeks.

Herpetiform ulcerations are less common that aphthous lesions and can occur more than one at the same time. Shape and size are similar to a pin head.

The minor criteria are:

■ Recurrent genital ulceration: aphthous ulceration or scarring observed by physician or patient.

■ Eye lesion: anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp examination or retinal vasculitis observed by ophthalmologist.

■ Skin lesions: erythema nodosum observed by physician or patient, pseudofolliculitis or papulopustular lesions, or acneform nodules observed by physician in post-adolescent patients not on corticosteroid treatment.

■ Positive pathergy test (Behcetine test) read by physician 24-48 hours.

The following table introduces a concise version of the criteria:

Pseudofolliculitis is the presence of ingrown curved hairs that provoke foreign body reaction and inflammation, often in the area of the beard.

Erythema nodosum is the inflammation of the fat cell layer below the skin. The main symptom (with resolution in about 1 month) is the presence of tender, red nodules or lumps that occur in the area of the shin.

Anterior and/or posterior uveitis is the swelling and destruction caused by the inflammation of the eye tissue. In some cases, it can extend to the retina and the optic nerve and affect vision.

Retinal vasculitis represents the inflammation of the vascular branches of the retinal artery. This is a symptom of sarcoidosis or multiple sclerosis as well.

Result interpretation

Diagnosis is considered positive when the major criteria is met (oral aphthosis present) and at least 2 of the minor criteria are met. This is considered as sufficient to classify a patient as suffering from Behcet’s syndrome.

Original study

The original study conducted by the International Study Group for Behcet’s Disease collected data on 914 patients from 12 centres, situated in 7 countries, as follows: France, Iran, Japan, Tunisia, Turkey, UK and USA.

The sensitivity for this set of criteria is respectively 65.4%, 78.1%, 83.7% and the specificity is 99.2%, 98.8%, 89.5%.

Benefits & Limitations

Compared to its predecessors, this set of criteria is deemed to have an improved discriminatory performance.

On the other hand, Davatchi argues in 2012 that although it has excellent specificity, it lacks sensitivity when compared to newer criteria such as the ICBD (83.7% to 96.5%) that was created in 2006.

Behcet’s syndrome symptoms and diagnosis

As a tissue inflammatory condition, this syndrome affects several areas of the body concomitantly, including affectation of the lining of the mouth, stomach disorders, joint pain, genital manifestations or fatigue.

The specific cause is not known, with some consensus on genetic predisposition, although the syndrome is not directly present at birth.

The following table introduces some of the most common symptoms, that are known to become more severe in time (without treatment):

Beside physical examination and symptom assessment, diagnostic (often lengthy) consists of lesion biopsy (oral, tegument or genital), inflammation markers (ESR, CRP), MRI or lumbar puncture, amongst others.

Treatment protocol is focused on addressing inflammation and local symptomatology. Some examples of therapy include: high dose corticosteroid therapy, interferon alpha-2a for oral and genital ulcers, colchicines for erythema nodosum or anti TFN therapy for uveitis.

Original reference

International Study Group for Behcet’s Disease. Criteria for diagnosis of Behcet’s disease. Lancet. 1990; 335(8697):1078-80.

Other references

1. Davatchi F. Diagnosis/Classification Criteria for Behcet’s Disease. Patholog Res Int. 2012; 2012: 607921.

2. Davatchi F, Shahram F, Akbarian M, et al. Accuracy of existing diagnosis criteria for Behcet’s disease. In: Wechsler B, Godeau P, editors. Behcet`s Disease. Vol. 1037. Amsterdam, The Netherlands: 1993. pp. 225–228. (Excerpta Medica International Congress Series).

3. Yazici H, Yazici Y. Criteria for Behcet’s disease with reflections on all disease criteria. J Autoimmun. 2014; 48-49:104-7.