Behçet Disease — Retinal Vasculitis with Vascular Sheathing MCQ — NEET PG Practice Question | NEETPGAI
Behçet Disease — Retinal Vasculitis with Vascular Sheathing
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eye Ophthalmology
A 26-year-old Turkish man with a 5-year history of recurrent oral aphthous ulcers, genital ulcerations, and erythema nodosum presents with acute blurred vision and floaters in the left eye. HLA-B51 is positive. Dilated fundus examination reveals a hazy vitreous with scattered intraretinal flame hemorrhages, patchy retinal whitening, and the structure marked **A** — white perivascular sheathing along retinal veins. Fundus fluorescein angiography confirms capillary non-perfusion. Which of the following is the most appropriate URGENT management strategy for this sight-threatening manifestation?
A. High-dose IV methylprednisolone followed by oral prednisolone AND simultaneous initiation of azathioprine, cyclosporine, or early biologic therapy (infliximab/adalimumab)
B. Oral prednisolone monotherapy with periodic laser photocoagulation for areas of non-perfusion
C. Topical corticosteroid drops and oral prednisolone monotherapy with close outpatient follow-up in 2 weeks
D. Periocular triamcinolone injection combined with oral methotrexate as steroid-sparing agent
Explanation
Why "High-dose IV methylprednisolone followed by oral prednisolone AND simultaneous initiation of azathioprine, cyclosporine, or early biologic therapy" is right
The white perivascular sheathing along retinal veins (marked A) is the pathognomonic fundus sign of occlusive retinal vasculitis in Behçet disease. This finding, combined with capillary non-perfusion on FFA, indicates severe posterior segment involvement — a sight-threatening manifestation with up to 25% risk of legal blindness within 5 years if untreated. Per EULAR 2018 guidelines for severe ocular Behçet disease, management requires URGENT high-dose systemic corticosteroids (IV methylprednisolone 1 g/day × 3 days followed by oral prednisolone 1 mg/kg/day) PLUS SIMULTANEOUS initiation of a steroid-sparing immunosuppressant (azathioprine, cyclosporine) or early biologic therapy (infliximab or adalimumab as first-line) to prevent relapse and preserve vision. Monotherapy is insufficient for this severity.
Why each distractor is wrong
Topical corticosteroid drops and oral prednisolone monotherapy with outpatient follow-up: Topical therapy alone is inadequate for posterior segment vasculitis. Oral prednisolone monotherapy without a steroid-sparing agent is insufficient for severe retinal vasculitis and risks long-term steroid toxicity; EULAR guidelines mandate concurrent immunosuppression or biologic therapy.
Oral prednisolone monotherapy with periodic laser photocoagulation: While laser photocoagulation may be used for areas of non-perfusion, it is a secondary intervention, not primary management. Monotherapy without immunosuppression or biologics is inadequate and contradicts EULAR 2018 recommendations for severe ocular Behçet.
Periocular triamcinolone injection combined with oral methotrexate: Periocular steroid injection is used for anterior uveitis or intermediate uveitis, not for posterior retinal vasculitis with capillary non-perfusion. Methotrexate is not a first-line steroid-sparing agent for Behçet ocular disease; azathioprine, cyclosporine, and biologics (infliximab, adalimumab) are preferred per EULAR 2018.
High-YieldNEET PG
White perivascular sheathing in Behçet retinal vasculitis = urgent systemic corticosteroids + simultaneous biologic or immunosuppressive therapy to prevent blindness.
Hatemi G. EULAR 2018 update for the management of Behçet syndrome. Tugal-Tutkun I. Behçet uveitis 2023 review.
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