Infliximab for laryngopharyngeal involvement in Behçet's syndrome

Laryngopharyngeal involvement in Behçet's syndrome is rare but clinically significant because it may cause severe odynophagia, dysphagia, and airway compromise. Optimal treatment has not been established, and irreversible structural sequelae, including laryngopharyngeal stenosis and ulcer scarring, may occur. We describe two men with Behçet's syndrome and symptomatic laryngopharyngeal involvement who showed rapid improvement after infliximab administration following an insufficient response to corticosteroids. In case 1, a 25-year-old man was classified as having suspected Behçet's syndrome based on recurrent oral ulcers, pharyngeal ulceration, arthritis, folliculitis-like skin lesions, and previous episodes of abdominal pain. He developed severe pharyngeal pain and dysphagia due to ulcerative lesions extending from the posterior pharyngeal wall to the esophageal inlet. Intravenous prednisolone resulted in limited endoscopic and symptomatic improvement, whereas infliximab promptly relieved pain and restored oral intake, with no recurrence over 14 years of follow-up. In case 2, a 29-year-old man presented with recurrent fever, oral aphthae, genital ulcers, arthritis, erythema nodosum-like lesions, and pharyngeal and laryngeal mucosal lesions associated with dysphagia. His symptoms and laryngoscopic abnormalities improved promptly after infliximab administration following an inadequate response to prednisolone. These cases underscore the importance of considering early anti-tumor necrosis factor-α therapy in steroid-refractory or relapsing laryngopharyngeal Behçet's syndrome, because progression to irreversible structural complications, including stenosis and adhesion, may necessitate surgical intervention if inflammation is not controlled before permanent damage occurs.