29 March 2024 | Salvatore Lavalle, Sebastiano Caruso, Roberta Foti, Caterina Gagliano, Salvatore Cocuzza, Luigi La Via, Federica Maria Parisi, Christian Calvo-Henriquez, Antonino Maniaci
**Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review**
Behçet’s disease is a systemic inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and uveitis, affecting multiple organ systems. Diagnosis is primarily clinical due to the lack of definitive diagnostic tests, and management involves a multidisciplinary approach to control inflammation and manage symptoms. Current treatment strategies include corticosteroids, immunosuppressive agents, and biological therapies. The disease exhibits a higher prevalence along the Silk Road, suggesting a role of environmental and genetic factors. Despite significant progress, gaps remain in understanding its pathogenesis. Future research is needed to elucidate the disease’s pathophysiology and optimize treatment strategies.
**Key Points:**
- **Pathogenesis:** Behçet’s disease is believed to result from an abnormal immune response triggered by environmental agents in genetically susceptible individuals.
- **Clinical Features:** Recurrent oral and genital ulcers, skin lesions, ocular involvement, musculoskeletal involvement, gastrointestinal symptoms, and neurological complications are common.
- **Epidemiology:** Prevalence is higher in countries along the Silk Road, with Turkey having the highest rates.
- **Diagnosis:** Based on clinical criteria, including oral ulcers and at least two of four key findings (genital ulcers, eye lesions, skin lesions, or positive pathergy test).
- **Treatment:** Management aims to reduce symptoms, prevent flare-ups, and manage complications. Treatments include corticosteroids, immunosuppressive agents, and biological therapies.
- **Prognosis:** Varies widely depending on severity and location of symptoms. Most patients can lead normal lives with appropriate treatment.
- **Complications:** Can include blindness, neurological damage, vascular events, and gastrointestinal complications.
- **Lifestyle Modifications:** Regular exercise, a balanced diet, stress management, and avoiding triggers can help manage the condition.
- **Mental Health:** Psychological support is crucial due to the chronic and unpredictable nature of the disease.
- **Pregnancy and Fertility:** Fertility is generally preserved, but close monitoring and medication management are essential during pregnancy.
**Conclusion:**
While significant progress has been made in understanding and managing Behçet’s disease, gaps in knowledge remain. Future research should focus on elucidating the pathogenesis, developing biomarkers, and optimizing treatment strategies to improve patient outcomes.**Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review**
Behçet’s disease is a systemic inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and uveitis, affecting multiple organ systems. Diagnosis is primarily clinical due to the lack of definitive diagnostic tests, and management involves a multidisciplinary approach to control inflammation and manage symptoms. Current treatment strategies include corticosteroids, immunosuppressive agents, and biological therapies. The disease exhibits a higher prevalence along the Silk Road, suggesting a role of environmental and genetic factors. Despite significant progress, gaps remain in understanding its pathogenesis. Future research is needed to elucidate the disease’s pathophysiology and optimize treatment strategies.
**Key Points:**
- **Pathogenesis:** Behçet’s disease is believed to result from an abnormal immune response triggered by environmental agents in genetically susceptible individuals.
- **Clinical Features:** Recurrent oral and genital ulcers, skin lesions, ocular involvement, musculoskeletal involvement, gastrointestinal symptoms, and neurological complications are common.
- **Epidemiology:** Prevalence is higher in countries along the Silk Road, with Turkey having the highest rates.
- **Diagnosis:** Based on clinical criteria, including oral ulcers and at least two of four key findings (genital ulcers, eye lesions, skin lesions, or positive pathergy test).
- **Treatment:** Management aims to reduce symptoms, prevent flare-ups, and manage complications. Treatments include corticosteroids, immunosuppressive agents, and biological therapies.
- **Prognosis:** Varies widely depending on severity and location of symptoms. Most patients can lead normal lives with appropriate treatment.
- **Complications:** Can include blindness, neurological damage, vascular events, and gastrointestinal complications.
- **Lifestyle Modifications:** Regular exercise, a balanced diet, stress management, and avoiding triggers can help manage the condition.
- **Mental Health:** Psychological support is crucial due to the chronic and unpredictable nature of the disease.
- **Pregnancy and Fertility:** Fertility is generally preserved, but close monitoring and medication management are essential during pregnancy.
**Conclusion:**
While significant progress has been made in understanding and managing Behçet’s disease, gaps in knowledge remain. Future research should focus on elucidating the pathogenesis, developing biomarkers, and optimizing treatment strategies to improve patient outcomes.