Regression of bilateral orbital inflammation with anti-CD20 in a patient with relapsing granulomatosis with polyangiitis: A case report

Purpose: To describe a case of bilateral orbital inflammation in a patient with relapsing granulomatosis with polyangiitis as only sign of disease recurrency treated with anti-CD20 antibodies. Methods: A 62-year-old Caucasian man affected by Granulomatosis with polyangiitis (GPA) was admitted to our hospital showing bilateral orbital inflammation as the only signs of disease recurrency. In addition, eye visit detected severe visual loss in the left eye (light perception). Guidelines to manage severe orbital involvement in patients with GPA are lacking. The patient was treated with intravenous rituximab and glucocorticoids. Results: Complete regression of inflammatory signs by imaging were observed at three-year of follow-up after the treatment with anti-CD20. However, ocular multimodal imaging showed severe optic nerve damages in the left eye with irreversible visual loss. Conclusion: Patients affected by GPA with inflammatory orbital involvement may benefit from anti-CD20 antibodies.