Avacopan rescue for Granulomatosis with Polyangiitis associated scleritis and panuveitis failing rituximab and cyclophosphamide

Purpose

To report the use of avacopan to achieve sustained steroid-free remission in a patient with ocular inflammation secondary to Granulomatosis with Polyangiitis (GPA) failing rituximab and cyclophosphamide.

Design

Case report

Participants

A patient with GPA-associated ocular disease failing rituximab and cyclophosphamide

Results

A 45-year-old man with a diagnosis of GPA developed nodular scleritis in the right eye. He was initially treated with steroids and rituximab with resolution of his scleritis. However, his scleritis recurred and progressed with development of panuveitis, which failed treatment with repeat rituximab dosing. All findings resolved two months after the addition of Avacopan.

Conclusion

This case report documents the second successful use of avacopan to induce steroid-free remission in a GPA patient who failed standard treatment with corticosteroids and rituximab.