From the Guidelines
Esophageal ulcerations in Behçet's disease should be managed with systemic corticosteroids, such as prednisone, as the first line of treatment, followed by immunosuppressants like azathioprine or TNF-alpha inhibitors for maintenance therapy. Esophageal ulcerations in Behçet's disease are less common than oral and genital ulcers but can cause significant symptoms including dysphagia, odynophagia, and chest pain. According to the EULAR recommendations for the management of Behçet disease 1, there is no evidence-based treatment specifically for gastrointestinal involvement, but agents such as sulfasalazine, corticosteroids, azathioprine, TNF-alpha antagonists, or thalidomide should be tried before surgery, except in emergencies.
Some key considerations for treatment include:
- Systemic corticosteroids, such as prednisone, at a dose of 0.5-1 mg/kg/day for acute flares, followed by gradual tapering over several weeks
- Immunossuppressants like azathioprine (2-3 mg/kg/day) for maintenance therapy
- TNF-alpha inhibitors, such as infliximab (5 mg/kg at weeks 0,2,6, then every 8 weeks) or adalimumab (40 mg every other week), may be necessary for some patients
- Topical treatments, like sucralfate suspension, can provide symptomatic relief
- Proton pump inhibitors, such as omeprazole, should be added to reduce acid-related damage
It is essential to note that the treatment of esophageal ulcerations in Behçet's disease aims to promote mucosal healing, prevent stricture formation or perforation, and reduce symptoms, with the ultimate goal of improving the patient's quality of life and reducing morbidity and mortality. Regular endoscopic follow-up is recommended to monitor healing and detect complications 1.
From the Research
Esophageal Ulcerations in Behcet's Disease
- Esophageal involvement in Behcet's disease is generally considered to be very uncommon, with only six cases of esophageal ulcers associated with perforation, penetration, or fistula described in the English literature 2.
- A case report described a patient with intestinal Behcet's disease who developed an esophageal ulcer with esophagobronchial fistula, which required endoscopic treatment and surgical intervention 2.
- The patient's symptoms gradually resolved without treatment, but recurred four years later with gastrointestinal hemorrhage and polyarthritis, and were successfully treated with steroid therapy 2.
Characteristics of Esophageal Ulcerations
- Volcano-shaped ulcers in the ileocecum are a typical finding of intestinal Behcet's disease, and punched-out ulcers can be observed in the intestine or esophagus 3.
- Esophageal ulcerations in Behcet's disease can be associated with complications such as perforation, penetration, or fistula 2.
Treatment of Esophageal Ulcerations
- Steroid therapy has been shown to be effective in treating esophageal ulcerations in Behcet's disease 2.
- Proton pump inhibitors have been used to treat esophageal ulcerations, but their use is not specifically recommended for Behcet's disease 4, 5, 6.
- Tumor necrosis factor inhibitors have been approved for the treatment of intestinal Behcet's disease and may be considered for the treatment of esophageal ulcerations 3.