Behçet-like Syndromes: A Comprehensive Review.

Area of Science:

• Rheumatology
• Immunology
• Genetics

Background:

• Behçet-like syndrome (BLS) encompasses Behçet's disease (BD) features associated with distinct conditions like immune defects, infections, or drug exposure.
• BLS may present differently from BD and is increasingly recognized as a separate clinical entity.
• Differentiating BLS from primary BD is vital for effective patient management and offers insights into BD pathogenesis.

Purpose of the Study:

• To systematically review and summarize the clinical features, treatment modalities, and genetic abnormalities reported in Behçet-like syndrome.
• To consolidate existing knowledge on BLS through a comprehensive analysis of published literature up to January 2024.

Main Methods:

• A systematic literature search was conducted across PubMed, Scopus, and Embase using relevant keywords for Behçet-like syndrome.
• Inclusion criteria involved English-language reports of patients over 12 years old with a defined etiology and BD-like manifestations (e.g., recurrent ulcers, gastrointestinal involvement, thrombosis).
• Data on epidemiology, clinical presentation, laboratory findings, histology, and treatments were extracted and descriptively analyzed.

Main Results:

• The review identified 100 patients with BLS from 53 publications, with a median age of 44 years and female predominance.
• Genetic disorders were identified in 70% of cases; common manifestations included skin lesions (68%), fever (56%), and intestinal involvement (43%).
• Treatments comprised glucocorticoids (65%), DMARDs (32%), biologics (22%), and in some cases, antiviral/antibiotic therapy or chemotherapy.

Conclusions:

• Various conditions, including monogenic diseases, immune deficiencies, myeloproliferative disorders, infections, and drug reactions, can manifest as Behçet-like features.
• Clinical presentation and treatment responses vary significantly across different etiologies of BLS.
• Identifying BLS is essential for appropriate management and advancing the understanding of BD pathogenesis and potential targeted therapies.