Treatment of Behçet's Disease

General Principles

The European League Against Rheumatism recommends that treatment for Behçet's disease should be individualized according to the type and severity of organ involvement, with the goal of promptly suppressing inflammatory exacerbations to prevent irreversible organ damage 1
Behçet's disease typically runs a relapsing and remitting course, with disease manifestations often ameliorating over time, according to the European League Against Rheumatism 1, 2
A multidisciplinary approach is necessary for optimal care due to the variety of organ systems that may be affected, as recommended by the European League Against Rheumatism 1
Ocular, vascular, neurological, and gastrointestinal involvement are associated with poor prognosis and require more aggressive treatment, according to the European League Against Rheumatism 1

The European League Against Rheumatism recommends first-line treatment for oral and genital ulcers: topical measures such as local corticosteroids 1, 3
For prevention of recurrent mucocutaneous lesions, especially when erythema nodosum or genital ulcers are dominant, the European League Against Rheumatism suggests colchicine should be tried first 1, 3, 4
For resistant mucocutaneous manifestations, the European League Against Rheumatism recommends considering azathioprine 1, 3 and thalidomide (with caution due to teratogenicity and peripheral neuropathy risk) 1, 3

The European League Against Rheumatism recommends that any patient with Behçet's disease and inflammatory eye disease affecting the posterior segment should be on a treatment regimen including azathioprine and systemic glucocorticoids (only in combination with immunosuppressives) 5, 6, 2
For severe eye disease, the European League Against Rheumatism suggests cyclosporine-A or infliximab in combination with azathioprine and corticosteroids 5, 6
For acute sight-threatening uveitis, the European League Against Rheumatism recommends high-dose glucocorticoids, infliximab, or interferon-alpha 2

The European League Against Rheumatism recommends immunosuppressive agents such as corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A for acute deep vein thrombosis 5, 6
For pulmonary and peripheral arterial aneurysms, the European League Against Rheumatism suggests cyclophosphamide and corticosteroids 5, 6

The European League Against Rheumatism recommends corticosteroids (high-dose pulsed, usually 3-7 pulses of IV methylprednisolone 1 mg/day) for parenchymal involvement 5, 7
For dural sinus thrombosis, the European League Against Rheumatism suggests corticosteroids 5, 7
The European League Against Rheumatism notes that cyclosporine A should not be used in patients with CNS involvement due to potential neurotoxicity 5, 7

The European League Against Rheumatism recommends trying medical therapy before considering surgery (except in emergencies) for gastrointestinal involvement, with options including sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, and thalidomide 5, 4

The European League Against Rheumatism suggests that in most patients, arthritis can be managed with colchicine 5, 3
Alternative options for joint involvement include indomethacin and benzathine penicillin 7

The European League Against Rheumatism recommends that treatment should be more aggressive in men with early age of disease onset due to typically more severe disease course 1
The European League Against Rheumatism notes that as disease manifestations usually abate over time, treatment may be tapered and even stopped during the course of the disease 1
The ultimate goal of treatment is to induce and maintain remission, preventing irreversible organ damage, according to the European League Against Rheumatism 1, 2