What is the recommended treatment plan for a patient with Behcet's disease?

Treatment of Behçet's Disease

Treatment of Behçet's disease must be tailored to the specific organ system involved, with aggressive immunosuppression required for sight-threatening, vascular, neurological, and gastrointestinal manifestations to prevent irreversible damage and death. 1

Organ-Specific Treatment Algorithms

Mucocutaneous Involvement (Oral/Genital Ulcers)

First-line approach:

• Start with topical corticosteroids applied directly to ulcers 2, 1
• Add colchicine 1-2 mg/day for prevention of recurrent lesions, particularly when erythema nodosum or genital ulcers dominate the clinical picture 2, 1

For resistant cases:

• Escalate to azathioprine 1
• Consider thalidomide, but only after careful counseling about teratogenicity and peripheral neuropathy risk 1

Eye Involvement (Posterior Uveitis/Retinal Disease)

Any posterior segment involvement requires immediate aggressive therapy:

• Azathioprine PLUS systemic corticosteroids (never use corticosteroids alone) 2, 1

For severe eye disease (>2 lines visual acuity drop or retinal vasculitis/macular involvement):

• Add either cyclosporine-A OR infliximab to the azathioprine-corticosteroid combination 2, 1
• Alternatively, use interferon-alpha with or without corticosteroids 2, 1

For acute sight-threatening uveitis:

• High-dose glucocorticoids PLUS infliximab or interferon-alpha immediately 1

Vascular Involvement

For acute deep vein thrombosis:

• Use immunosuppressive agents: corticosteroids combined with azathioprine, cyclophosphamide, or cyclosporine-A 2, 1
• Do NOT use anticoagulants, antiplatelet agents, or antifibrinolytic drugs - venous thrombi in Behçet's adhere to vessel walls and rarely embolize, and anticoagulation risks fatal bleeding from coexisting pulmonary arterial aneurysms 2

For pulmonary or peripheral arterial aneurysms:

• Cyclophosphamide PLUS corticosteroids 2, 1

Neurological Involvement (Neurobehçet's)

For parenchymal CNS disease:

• High-dose IV methylprednisolone (1 g/day for 3-7 pulses) followed by oral prednisolone taper over 6-12 months 1, 3
• Simultaneously start azathioprine 2.5 mg/kg/day as steroid-sparing agent (methotrexate 15-25 mg/week is alternative) 3

For dural sinus thrombosis:

• High-dose corticosteroids 2, 1, 3

Critical contraindication:

• Never use cyclosporine-A in CNS involvement - significant neurotoxicity risk 2, 1, 3

For refractory cases:

• TNF-alpha inhibitors, particularly infliximab 3

Gastrointestinal Involvement

Medical therapy before surgery (except emergencies):

• Try sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide before considering surgical intervention 2, 1
• Azathioprine decreases re-operation rates and should be used as maintenance therapy in patients requiring surgery 2

Joint Involvement (Arthritis)

First-line:

• Colchicine 1-2 mg/day - effective for the typically mild, transient, non-erosive arthritis affecting large joints 2, 1

Alternatives if colchicine fails:

• Indomethacin or benzathine penicillin 1

Critical Clinical Pitfalls

Young males with early disease onset:

• Require more aggressive treatment from the start due to typically more severe disease course 1, 3

Pulmonary arterial aneurysms:

• Must be ruled out before starting anticoagulation for DVT - risk of fatal hemorrhage 4

Corticosteroid monotherapy:

• Never use systemic corticosteroids alone for posterior uveitis - must combine with immunosuppressives 4

Delayed treatment:

• Sight-threatening uveitis, neurological disease, and arterial involvement cause irreversible damage if treatment is delayed 4

Disease Course and Treatment Adjustment

• Behçet's disease typically follows a relapsing-remitting course with manifestations often ameliorating over time 1, 3
• Treatment may be tapered and potentially discontinued during disease quiescence 1, 3
• Multidisciplinary approach involving rheumatology, ophthalmology, neurology, gastroenterology, and vascular surgery is essential 1