Behçet's disease diagnostic study of choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

Currently, there is not a specific test to confirm the diagnosis of Behçet's disease. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks obtained by a thorough history of the patient's symptoms (outlined below). Behcet's disease is a diagnosis of exclusion, and other chronic inflammatory diseases should be evaluated for. The various inflammatory symptoms do not necessarily occur together, and they will vary in severity.

Diagnostic Study of Choice

Study of choice

• The pathergy test should be performed when:
  • The patient presented with symptoms of vision problems, mouth sores, and genital ulcers
  • A positive [test] is detected in the patient.
• Behcet disease is mainly diagnosed based on clinical presentation.

Diagnostic Criteria

There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is made on the basis of the clinical findings. In the absence of other systemic diseases, we diagnose Behçet syndrome in patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features:

●Recurrent genital aphthae (aphthous ulceration or scarring).

●Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis observed by an ophthalmologist).

●Skin lesions (including erythema nodosum, pseudo-vasculitis, papulopustular lesions, or acneiform nodules consistent with Behçet syndrome).

●A positive pathergy test. Pathergy is defined by a papule 2 mm or more in size developing 24 to 48 hours after oblique insertion of a 20-gauge needle 5 mm into the skin, generally performed on the forearm.

This approach is consistent with the International Study Group (ISG) diagnostic criteria published in 1990 (table 2) [25]. These remain the most widely used and well-accepted criteria among experts in Behçet syndrome. (See 'Classification criteria' below.)

Pathergy is less common in Northern European and North American patients. Thus, it has been suggested that other features might be substituted for pathergy in these populations, including aseptic meningoencephalitis, cerebral vasculitis, recurrent phlebitis, arteritis, synovitis, or focal bowel ulceration [106].

There are patients who do not meet these criteria in whom the diagnosis of Behçet syndrome is still made, and establishing the diagnosis in such patients is much more difficult. Thus, it is often appropriate to refer a patient in whom the diagnosis of Behçet syndrome is suspected to a rheumatologist with experience in this disease.

Diagnostic Criteria

There are three levels of certainty for diagnosis:

1. International Study Group diagnostic guidelines (very strict for research purposes)
2. Practical clinical diagnosis (generally agreed pattern but not as strict)
3. 'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)

International Study Group diagnostic guidelines

The ISGD guidelines recommend the following:

• Must have oral (aphthous) 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 dia. 24-48 hrs or more after needle-prick).

Practical clinical diagnosis

Must have

• mouth ulcers,

along with 1 of the 4 hallmark symptoms above and with 2 of the symptoms below:

• arthritis/arthralgia,
• nervous system symptoms,
• stomach and/or bowel inflammation,
• deep vein thrombosis,
• superficial thrombophlebitis,
• cardio-vascular problems of inflammatory origin,
• inflammatory problems in chest and lungs,
• problems with hearing and/or balance,
• extreme exhaustion,
• changes of personality, psychoses,
• any other members of the family with a diagnosis of Behcet disease.

'Suspected' or 'Possible' diagnosis

Usually assigned 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.