What is the recommended management approach for an elderly patient with Behçet’s disease, including assessment of organ involvement and age‑related considerations?

Management of Elderly Patients with Behçet's Disease

Elderly patients with Behçet's disease require a multidisciplinary approach with systematic assessment of organ involvement, prompt immunosuppressive therapy tailored to affected systems, and careful consideration of age-related comorbidities and medication tolerability.

Initial Assessment and Organ System Evaluation

When evaluating an elderly patient with Behçet's disease, systematically assess for involvement of each major organ system, as the disease follows a relapsing-remitting course requiring prompt suppression of inflammatory exacerbations to prevent irreversible organ damage 1.

Critical Organ Systems to Evaluate:

Ocular involvement (70-85% of patients):

• Assess for anterior uveitis, hypopyon, and posterior segment involvement including retinal vasculitis 2
• Any posterior segment inflammation mandates immediate treatment with azathioprine (2.5 mg/kg/day) combined with systemic corticosteroids, as corticosteroids alone are contraindicated 3
• Severe eye disease (>2 lines visual acuity drop or retinal disease) requires escalation to either infliximab or cyclosporine A in addition to azathioprine and corticosteroids 3

Neurological involvement (5-10% of cases):

• Distinguish between parenchymal neuro-Behçet's (brainstem syndrome, hemiparesis) and extra-axial disease (cerebral venous sinus thrombosis) 4, 5
• MRI shows ill-defined T2-FLAIR hyperintensities in basal ganglia or brainstem with possible swelling and hemorrhage 5
• Critical caveat: Avoid cyclosporine A entirely if any CNS involvement is present or suspected due to significant neurotoxicity risk 3

Vascular involvement (one-third of patients):

• Assess for venous thrombosis (superficial thrombophlebitis, deep vein thrombosis, cerebral sinus thrombosis) and arterial complications (aneurysms, stenosis) 1, 6
• Aortic involvement shows lymphocytic infiltration with destruction of media leading to aneurysm and potential rupture 1

Gastrointestinal involvement:

• Look for ulcerations (particularly ileocecal region), enteric fistulas, and deeply penetrating ulcers 6

Mucocutaneous and joint manifestations:

• Oral ulcers (required for diagnosis), genital ulcers, erythema nodosum, pseudofolliculitis, pathergy 1
• Arthritis typically affects large joints (knees, ankles) without deformities or erosions 3

Treatment Algorithm Based on Disease Severity

Mild Mucocutaneous and Joint Disease:

First-line therapy:

• Topical corticosteroids for isolated oral and genital ulcers 3
• Colchicine 1-2 mg/day for erythema nodosum, mild mucocutaneous lesions, and arthritis (particularly effective in women) 3, 7

Refractory cases:

• Consider azathioprine, interferon-alpha, or TNF-alpha antagonists 3

Severe Organ Involvement (Eye, Neurological, Vascular, GI):

Initial high-dose corticosteroid regimen:

• Start with oral prednisolone 1 mg/kg/day for acute attacks 3
• For severe presentations (posterior uveitis, parenchymal neuro-Behçet's), use intravenous methylprednisolone pulses 1 g/day for 3-7 days, followed by oral prednisolone 1 mg/kg/day 3, 5

Mandatory steroid-sparing immunosuppression:

• Azathioprine 2.5 mg/kg/day is the cornerstone immunosuppressant for severe organ involvement 3, 7
• Begin tapering prednisolone by 5-10 mg every 10-15 days after clinical response, aiming for maintenance dose of 5-10 mg/day 3
• Continue maintenance therapy for at least 2 years and at least 12 months after normalization of inflammatory markers 3

Escalation for refractory or severe disease:

• Cyclophosphamide (pulse therapy) combined with corticosteroids for severe systemic vasculitis, pulmonary/peripheral arterial aneurysms 3, 7
• Infliximab for refractory eye disease (rapid response in 1-5 days) or severe cases, though screen for tuberculosis first as endemic areas overlap 3
• Interferon-alpha for refractory ocular and extraocular manifestations 7

Specific Organ-Based Protocols:

Acute deep vein thrombosis:

• Use immunosuppressive agents (corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A) 3
• Avoid anticoagulants due to bleeding risk, especially with potential coexisting pulmonary arterial aneurysms 3

Parenchymal neuro-Behçet's:

• High-dose glucocorticoids followed by slow taper with azathioprine for long-term prevention 3, 4
• Severe forms may require intravenous cyclophosphamide or infliximab 5

Cerebral venous thrombosis:

• High-dose glucocorticoids followed by tapering 3

Gastrointestinal involvement:

• Trial sulfasalazine, corticosteroids, azathioprine before considering surgery (except emergencies) 3
• TNF-alpha antagonists and/or thalidomide for severe/refractory cases 3

Age-Related Considerations in Elderly Patients

Medication Tolerability and Monitoring:

Corticosteroid complications:

• Provide calcium and vitamin D supplementation to all patients on corticosteroids 3
• Perform bone DEXA scanning at 1-2 yearly intervals 3
• Monitor for hyperglycemia, hypertension, and mood changes 3
• If steroid-induced diabetes develops, coordinate with endocrinology for glycemic control, as uncontrolled hyperglycemia worsens retinal disease 3

Azathioprine monitoring:

• Regular dermatologic surveillance for skin changes or new lesions given increased malignancy risk 8
• Coordinate with rheumatology to ensure optimal disease control 8

Cyclosporine considerations:

• Monitor for renal dysfunction, hypertension, and nephrotoxicity 3
• Absolute contraindication if any CNS involvement 3

Cardiovascular Risk Management:

Elderly patients with Behçet's disease have compounded cardiovascular risk from both systemic inflammation and age-related factors 9.

Optimal control of underlying Behçet's inflammation is the most critical intervention for reducing cardiovascular risk, as systemic inflammation drives endothelial dysfunction and atherogenesis 9.

Lipid management thresholds:

• Initiate statin therapy if LDL-C ≥190 mg/dL regardless of other risk factors 9
• Consider statins if LDL-C ≥160 mg/dL with multiple cardiovascular risk factors (hypertension, diabetes, smoking, family history) 9
• Omega-3 fatty acids (EPA 1800 mg/day) may be considered if triglycerides ≥150 mg/dL or HDL-C <50 mg/dL 9

Lifestyle interventions:

• Maintain BMI 18.5-24.9 kg/m² and waist circumference <35 inches 9
• At least 150 minutes per week of moderate-intensity aerobic exercise 9
• Limit saturated fat to <7% of calories and cholesterol to <200 mg/day if hypercholesterolemia develops 9

Monitoring and Follow-Up

Regular assessments:

• Clinical symptoms and inflammatory markers (ESR, CRP) 3
• Repeat imaging for neurological or vascular involvement 3
• Regular ophthalmologic examinations for ocular involvement 3
• If inadequate response after 3 days of high-dose steroids, consider early introduction of biologics such as infliximab 3

Common Pitfalls to Avoid

• Never use corticosteroids alone for posterior uveitis—always combine with azathioprine 3
• Avoid cyclosporine A if any neurological involvement is present or suspected 3
• Do not use anticoagulants for venous thrombosis in Behçet's disease due to bleeding risk from potential arterial aneurysms 3
• Post-thrombotic syndrome is frequent with recurrent DVT and may result in difficult-to-treat leg ulcers 3
• Surgery for Behçet's disease has high complication and recurrence rates; medical management should be exhausted first except in emergencies 6