To characterize the clinical features of patients with ocular inflammatory diseases who tested positive for atypical perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA).

Retrospective case series of patients with ocular inflammatory disease seen at the Massachusetts Eye Research and Surgery Institute (MERSI) from April 2014 to April 2016 who were tested for ANCA.

Eight hundred and thirteen patients were tested for ANCA with 34 patients (4%) being positive for atypical P-ANCA. Among these patients who were positive for atypical P-ANCA, the most frequent diagnoses were anterior uveitis followed by scleritis in 62% (n=21) and 20% (n=7), respectively. Three patients had systemic vasculitis; two of which were positive for typical C-ANCA and P-ANCA in addition to atypical P-ANCA. The third patient had leukocytoclastic vasculitis. Ocular presentation in all cases of atypical P-ANCA with systemic vasculitis was scleritis. Only one patient in study cohort had an episode of recurrent disease flare-up. All but one patient, who had concomitant C-ANCA seropositivity and granulomatosis with polyangiitis, had a favorable disease course with control of their intraocular inflammation with topical therapy and or systemic immunomodulatory therapy.

In contrast to typical c-ANCA and p- ANCA, atypical P-ANCA seropositivity was not associated with severe vasculitis or poor prognosis in patients with the ocular inflammatory diseases.