Screening and diagnosis

Classification and risk stratification

Perioperative care

Quality improvement

Patient education

Nonpharmacologic interventions

Therapeutic procedures

Respiratory support

Follow-up and surveillance

Specific circumstances

Preventative measures

Medical management

Surgical interventions

Inpatient care

Diagnostic procedures

Diagnostic investigations

Insufficient evidence to make a recommendation.

Strongly favor this test or intervention.

This diagnostic tool assesses the likelihood of Behçet's syndrome by examining five key clinical features, each contributing to an overall score. 

- Recurrent oral ulcers, whether major, minor aphthous, or herpetiform, occurring three or more times per year, are a significant indicator, contributing 5 points if present.
- Recurrent genital ulcers, characterized by aphthous or scarring lesions, add 1 point to the total score.
- Eye disorders, including anterior or posterior uveitis, cells in the vitreous identified through slit lamp exam, or retinal vasculitis, contribute another 1 point.
- Skin manifestations such as erythema nodosum, pseudofolliculitis, papulopustular lesions, or specific acneiform nodules earn 1 point. These are relevant for post-adolescent patients not on steroids.
- A positive pathergy test, evaluated at 24-28 hours, is also worth 1 point.

Each symptom is checked and given its corresponding score, and the total is then calculated by summing these individual scores.

To interpret the results of this calculation:
- A cumulative score of 7 or higher indicates "Diagnostic criteria are met," supporting the diagnosis of Behçet's syndrome.
- A score below 7 means "Diagnostic criteria not met," suggesting that further criteria exploration is necessary to consider Behçet's syndrome diagnosis.

| Clinical Feature                                                    | Points if Present |
|---------------------------------------------------------------------|------------------|
| Recurrent oral ulcers                                               | 5                |
| Recurrent genital ulcers                                            | 1                |
| Eye lesions                                                         | 1                |
| Skin lesions                                                        | 1                |
| Positive pathergy test                                              | 1                |

This score should be used in conjunction with comprehensive clinical judgment and possibly further investigation.

The International Study Group (ISG) diagnostic criteria for Behçet's syndrome is a clinical calculator used to diagnose Behçet's syndrome, a rare and chronic multisystem inflammatory disorder. This calculator is applicable to patients presenting with symptoms suggestive of Behçet's syndrome, such as recurrent oral and genital ulcers, eye inflammation, and skin lesions.

The ISG criteria for Behçet's syndrome is clinically useful in identifying patients with this disorder, guiding treatment decisions, and facilitating research. The criteria include recurrent oral ulceration, plus at least two of the following: recurrent genital ulceration, eye lesions, skin lesions, or a positive pathergy test.

There are no specific exclusion criteria for the use of the ISG diagnostic criteria for Behçet's syndrome. However, it should be noted that these criteria are not intended to replace clinical judgment and should be used in conjunction with a comprehensive clinical evaluation. Other causes of the symptoms should be ruled out before a diagnosis of Behçet's syndrome is made.

International Study Group (ISG) diagnostic criteria for Behçet's syndrome

Behçet's Disease is evaluated using several clinical criteria, each contributing to a total score that aids in diagnosis. This process involves assessing seven symptoms, with each criterion assigned a certain number of points. Here’s how the calculation works:

- Oral aphthous ulcers: 2 points
- Genital aphthous ulcers: 2 points
- Ocular manifestations such as anterior or posterior uveitis, or retinal vasculitis: 2 points
- Skin lesions including pseudofolliculitis, skin aphthosis, or erythema nodosum: 1 point
- Neurologic manifestations: 1 point
- Vascular manifestations, such as arterial thrombosis, large vein thrombosis, generalized phlebitis, or superficial phlebitis: 1 point
- Positive pathergy test: 1 point

Patients provide information on these symptoms, indicating whether they are present or not. The calculated score ranges between a minimum of 0 and a maximum of 10. This is achieved by summing the points corresponding to the positive responses.

The interpretation of the score is direct and crucial for diagnosis:
- A score of 4 or more means that the diagnostic criteria for Behçet's Disease are met. This suggests a strong possibility of the presence of Behçet's Disease, warranting further medical evaluation.
- A score below 4 indicates that the diagnostic criteria for Behçet's Disease are not met, suggesting that the condition is less likely based on the chosen criteria.

The calculator provides both the total score and the interpretation to help clinicians and patients understand the likelihood of having Behçet's Disease.

The International Criteria for Behçet's Disease (ICBD) is a clinical scoring system used to diagnose Behçet's Disease, a rare and chronic multisystem inflammatory disorder. This calculator is applicable to patients presenting with symptoms suggestive of Behçet's Disease, such as recurrent oral and genital ulcers, eye inflammation, skin lesions, and other systemic manifestations.

The ICBD is clinically useful in standardizing the diagnosis of Behçet's Disease across different populations and clinical settings. It helps clinicians to make a more accurate diagnosis, guide treatment decisions, and predict disease course and prognosis.

The ICBD includes oral aphthosis, genital aphthosis, ocular lesions, skin lesions, and a positive pathergy test as diagnostic criteria. Each of these criteria is scored, and a certain score threshold must be met for a diagnosis of Behçet's Disease.

There are no specific exclusion criteria for the use of the ICBD. However, it should be noted that this scoring system is not intended to replace clinical judgment. Other differential diagnoses should be considered and ruled out before confirming a diagnosis of Behçet's Disease based on the ICBD score.

International Criteria for Behçet's Disease (ICBD)

Behçet's syndrome

Management of arthritis

Management of arthritis, immunosuppressants

Management of gastrointestinal involvement

Management of gastrointestinal involvement, resistant disease

Management of arterial involvement

Management of arterial involvement, surgery

Management of venous thrombosis

Management of central nervous system involvement

Management of CNS involvement, pharmacotherapy

Diagnostic criteria

Prophylaxis for Pneumocystis jirovecii pneumonia

Prophylaxis for P. jirovecii pneumonia

Assessment of quality of life

Assessment of QoL

Psychological support

Transition to adult care

Management of arterial involvement, pharmacotherapy

Management of arthritis (specialist referral)

Clinical follow-up

Management of arthritis, colchicine

Management of mucocutaneous lesions

Management of mucocutaneous lesions, systemic therapy

Management of mucocutaneous lesions, topical therapy

Management of arthritis, corticosteroids

Management of CNS involvement (evaluation)

Management of gastrointestinal involvement (evaluation)

General principles

Pregnant patients

Management of arthritis (NSAIDs)

Pretreatment evaluation

Management of gastrointestinal involvement, corticosteroids

Management of uveitis

Assessment of disease activity

Management of gastrointestinal involvement (surgery)

General counseling

Routine immunizations

Management of gastrointestinal involvement (immunosuppressants)

Updated 2024 BAD/BSR guildeines for the diagnosis and management of Behçet's syndrome


Behçet's syndrome

Background

Overview

Guidelines

Key sources