Fig. 2.

a) Necrotizing scleritis of the right eye at presentation in acute phase. The patient (number 9 of Table 1), who was c-ANCA-negative and p-ANCA-positive, was treated with a combination of GC plus CYC, which resulted in total regression of the scleritis and a substantial reduction of the pulmonary nodules. b) Left eye in a woman (number 10 of Table 1) at presentation with active necrotizing scleritis OU and multi-organ GPA. She was given six infusions of CYC and four infusions of RTX intravenously. Her scleritis, which appeared while she was on maintenance therapy with MMF plus GC, resolved with the addition of CYC. c), d) Active necrotizing scleritis of the right eye in a woman (number 11 of Table 1) with severe lung involvement, showing scleral melt and uveal prolapse superiorly (2c). Two doses of RTX were administered to control inflammation, followed by a scleral patch graft (2d) and maintenance therapy consisting of azathioprine, MMF, and GC. At the patient’s last follow-up, her scleritis had resolved, the graft had integrated, and her eye condition was considered stable