# Behçet-like Syndromes: A Comprehensive Review

## TL;DR

This review summarizes Behçet-like syndromes, which mimic Behçet’s disease but stem from other conditions like genetic defects or infections, emphasizing the need for accurate diagnosis and treatment.

## Contribution

The study compiles and analyzes all published cases of Behçet-like syndromes up to 2024, revealing distinct clinical and genetic patterns that differentiate them from Behçet’s disease.

## Key findings

- Behçet-like syndromes are associated with genetic disorders in 70% of cases and show distinct clinical features like skin lesions and intestinal involvement.
- Treatments vary by etiology, with glucocorticoids and anti-TNF biologics being most common, and allogeneic stem cell transplantation used in some myeloproliferative cases.
- The study highlights the importance of distinguishing Behçet-like syndromes from Behçet’s disease to guide appropriate therapeutic strategies.

## Abstract

Behçet-like syndromes (BLSs) are conditions that present with symptoms similar to Behçet’s disease—such as oral and genital ulcers, fever, skin lesions, joint pain, and intestinal involvement—but arise in association with other underlying disorders. These include monogenic immune defects, myeloproliferative diseases, infections, or reactions to specific medications. Because BLS can resemble Behçet’s disease but requires different treatments, recognizing the underlying cause is essential for proper management. In this review, we summarize all published cases of BLS up to January 2024, highlighting their clinical features, genetic findings, and therapeutic approaches. Understanding BLS as a distinct group of conditions may also provide insights into the mechanisms that drive Behçet’s disease itself.

Background: Behçet-like syndrome (BLS) refers to the presence of Behçet’s disease (BD) features occurring in association with distinct clinical–pathological conditions such as inborn errors of immunity, myeloproliferative disorders, infections, or drug exposure. BLS may differ clinically from BD and is increasingly recognized as a separate entity. Distinguishing BLS from primary BD is essential for appropriate management, and studying BLS may provide insights into BD pathogenesis. Objectives: To summarize clinical features, treatments, and genetic abnormalities reported in BLS, we reviewed all published cases up to January 2024. Methods: A systematic search of PubMed, Scopus, and Embase was performed using the terms “Behçet-like syndrome”, “Behçet-like disease”, and “Pseudo-Behçet disease”. We included English-language reports of patients > 12 years old with a defined underlying etiology and Behçet-like manifestations, defined by ≥2 ICBD criteria and/or gastrointestinal involvement, mucosal ulcers, thrombosis, or non-recurrent disease. Epidemiological, clinical, laboratory, histological, and treatment data were extracted and analyzed descriptively. Results: Of 679 publications, 53 met inclusion criteria, comprising 100 patients with BLS. The median age was 44 years (IQR 22–52), with a female predominance (1:2). Fifty-three percent were from non-European countries. A genetic disorder was identified in 70% of cases, while HLA-B51 was present in 10%. Frequent manifestations included skin lesions (68%), fever (56%), intestinal involvement (43%), and joint symptoms (43%). Treatments included glucocorticoids (65%), conventional DMARDs (32%), and biologics (22%), mainly anti-TNF agents. Antiviral/antibiotic therapy was used in 9% and chemotherapy in 15%. Two patients with trisomy-8 MDS underwent allogeneic stem cell transplantation. Conclusions: Diverse conditions—including monogenic diseases, immune defects, myeloproliferative disorders, infections, and drug-related reactions—can produce Behçet-like features. Our findings highlight differences in clinical expression and treatment response across BLS etiologies. Recognizing BLS is essential for appropriate management and may contribute to a deeper understanding of BD pathogenesis and future targeted therapies.