Atypical perinuclear anti-neutrophil cytoplasmic antibodies in ocular inflammatory diseases

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