Behçet’s disease (BD) is a chronic and recurrent systemic vasculitis of unclear origin. Clinical features involve mucocutaneous lesions, ocular, vascular, articular, gastrointestinal, urogenital, and neurologic manifestations. BD remains associated with severe morbidity and mortality, despite newer and more effective drugs. A more serious course is typically associated with male gender, a younger age of onset, and irregular treatment and follow-up. The disease is typically more severe in the early stages, but it tends to diminish in most patients after reaching their forties. The main treatment goal should be to suppress and prevent new inflammatory attacks rapidly, especially during the initial active stages of BD, in order to avoid irreversible organ damage. Thus, early diagnosis and appropriate treatment are crucial in reducing the morbidity and mortality of the disease. The treatment approach varies depending on which organs are affected and the severity of involvement. Therefore, treatment needs to be organized individually with a multidisciplinary approach. The mainstay of treatment involves suppressing the disease’s inflammatory attacks using a combination of local and systemic immunomodulatory and immunosuppressive drugs. This review suggests a stepwise, symptom-based, algorithmic approach for managing BD with a holistic perspective.

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Algorithmic Approach to the Treatment of Behçet’s Disease

  • Erkan Alpsoy

摘要

Behçet’s disease (BD) is a chronic and recurrent systemic vasculitis of unclear origin. Clinical features involve mucocutaneous lesions, ocular, vascular, articular, gastrointestinal, urogenital, and neurologic manifestations. BD remains associated with severe morbidity and mortality, despite newer and more effective drugs. A more serious course is typically associated with male gender, a younger age of onset, and irregular treatment and follow-up. The disease is typically more severe in the early stages, but it tends to diminish in most patients after reaching their forties. The main treatment goal should be to suppress and prevent new inflammatory attacks rapidly, especially during the initial active stages of BD, in order to avoid irreversible organ damage. Thus, early diagnosis and appropriate treatment are crucial in reducing the morbidity and mortality of the disease. The treatment approach varies depending on which organs are affected and the severity of involvement. Therefore, treatment needs to be organized individually with a multidisciplinary approach. The mainstay of treatment involves suppressing the disease’s inflammatory attacks using a combination of local and systemic immunomodulatory and immunosuppressive drugs. This review suggests a stepwise, symptom-based, algorithmic approach for managing BD with a holistic perspective.