Behcet's disease: from East to West.

Area of Science:

• Rheumatology
• Immunology
• Vasculitis Research

Background:

• Behcet's disease (BD) is a rare multisystem inflammatory disorder classified as a vasculitis.
• Historically prevalent along the Silk Route, BD is now observed globally, necessitating widespread epidemiological understanding.
• Existing nationwide surveys and large case series provide valuable data on BD's distribution and characteristics.

Purpose of the Study:

• To consolidate and analyze epidemiological data from nationwide surveys and case series of Behcet's disease.
• To describe the global distribution, demographic variations, and clinical manifestations of BD.
• To evaluate the utility of diagnostic criteria and laboratory tests in identifying Behcet's disease.

Main Methods:

• Systematic review and analysis of five nationwide surveys (Iran, Japan, China, Korea, Germany) and four large case series (Turkey, Morocco, Tunisia, UK).
• Data extraction included male to female ratios, mean age at onset, and prevalence of major and minor clinical manifestations.
• Evaluation of diagnostic accuracy for the International Criteria for Behcet's Disease (ICBD) and the pathergy test.

Main Results:

• Behcet's disease exhibits varying male to female ratios and mean age at onset across different surveyed populations.
• Mucous membrane involvement (oral aphthosis) is highly prevalent (97-99%) across all surveyed regions.
• Skin, ocular, joint, neurological, gastrointestinal, vascular, pulmonary, and cardiac manifestations show diverse prevalence rates.
• The pathergy test shows variable positivity (34-54%), and ESR is often normal; diagnosis primarily relies on clinical manifestations.
• The International Criteria for Behcet's Disease (ICBD) demonstrated high sensitivity (98.2%) and specificity (95.6%) in Iranian patients.

Conclusions:

• Behcet's disease is a globally distributed vasculitis with diverse epidemiological and clinical features.
• Clinical manifestations, particularly oral aphthosis, are key diagnostic indicators.
• The International Criteria for Behcet's Disease (ICBD) provides a reliable framework for diagnosis, supported by clinical assessment.