Behçet's disease secondary prevention

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2] Dheeraj Makkar, M.D.[3]

There are no established measures for the secondary prevention of Behçet disease.

There are no established measures for the secondary prevention of Behçet disease.

• General Principles

Aim is to control inflammation early and maintain remission to prevent organ damage.

Immunosuppressive therapy is central to relapse prevention.

Treatment must be individualized depending on organ involvement (mucocutaneous, ocular, vascular, neurologic, gastrointestinal).

• Mucocutaneous and Joint Disease

Colchicine: widely used for oral/genital ulcers and arthritis; helps reduce recurrence.

Topical corticosteroids: for oral/genital ulcers during flares.

Azathioprine or apremilast: options when colchicine is insufficient.

• Ocular Disease (Prevention of Vision Loss)

Azathioprine, cyclosporine, TNF inhibitors, or interferon-alpha are used to prevent relapses of uveitis and long-term blindness.

Early and aggressive therapy is recommended, particularly in young men with posterior uveitis or retinal vasculitis, who have the highest risk of vision loss.

• Vascular Disease

Immunosuppressive therapy (azathioprine, cyclophosphamide, TNF inhibitors) reduces risk of recurrence and catastrophic events such as pulmonary artery aneurysm rupture or Budd–Chiari syndrome.

Anticoagulation is controversial; immunosuppression is prioritized because vascular lesions are inflammatory rather than thrombotic in origin.

Secondary prevention of aneurysm rupture: immunosuppression before any surgical or endovascular repair.

• Neurologic Disease

High-dose corticosteroids for acute attacks, followed by immunosuppressive maintenance (azathioprine, cyclophosphamide, or TNF inhibitors) to reduce relapses and disability.

• Gastrointestinal Disease

Azathioprine, TNF inhibitors: prevent relapse of ulcerative lesions and reduce risk of perforation.

Surgery is reserved for emergencies; secondary prevention centers on ongoing immunosuppression to avoid recurrence at surgical sites.

• Biologic Therapies

TNF inhibitors (infliximab, adalimumab, etanercept): effective across multiple organ systems in preventing relapses.

Interleukin-1 and Interleukin-17 inhibitors: under investigation for refractory disease.

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