Adjunctive Orbital Radiotherapy for Ocular Adnexal IgG4-related Disease: Preliminary Experience in Patients Refractory or Intolerant to Corticosteroid Therapy

Abstract

Purpose: To present the clinical outcomes of combined orbital radiotherapy and systemic corticosteroid for patients with refractory ocular adnexal IgG4-related disease.

Methods: We retrospectively reviewed 3 patients with histopathologically confirmed ocular adnexal IgG4-related disease who had been refractory or intolerant to corticosteroid therapy and treated with adjunctive orbital radiotherapy (2000 cGy; 10 fractions). Clinical improvement was assessed by monitoring the patient's ability to taper corticosteroid to discontinuation and by follow-up radiologic examination.

Results: All 3 patients had a favorable response to adjunctive radiotherapy with improvement of the clinical symptoms and radiologic abnormalities. Systemic corticosteroid was tapered and discontinued in all patients successfully. There were no adverse effects of treatment or recurrence after a mean follow-up of 19 months.

Conclusion: Adjunctive radiotherapy can help to achieve stable disease and cessation of systemic corticosteroid in patients with refractory ocular adnexal IgG4-related disease.