Question

Necrotizing scleritis in a patient positive for both c- and p-anca without underlying systemic vasculitis

Answer 1

Scleritis is a painful inflammatory disorder of the outer coating of the eye. Any form of scleritis associated with a systemic disease is more likely to be aggressive [1], and it is now recognized that antineutrophil cytoplasmic antibody (ANCA)-associated active scleritis tends to have more severe inflammation and requires more aggressive therapy [2]. Here, we report a case of necrotizing scleritis positive for both cytoplasmic pattern ANCA and perinuclear staining pattern ANCA without underlying systemic vasculitis, which was successfully treated with intravenous pulse cyclophosphamide therapy.