Perinuclear Antineutrophil Cytoplasmic Antibody-Associated Ocular Manifestations: Case Series and Literature Review

ABSTRACT

Purpose

To investigate the diversity of ocular manifestations in patients with positive perinuclear antineutrophil cytoplasmic antibody (p-ANCA).

Methods

The clinical records of five patients exhibiting ocular manifestations and testing positive for serum MPO-ANCA were retrospectively reviewed.

Results

Three out of five patients were female. The youngest patient was aged 26, whereas the eldest was 83 years old. 80% (n = 4) of them had purely anterior segment involvement, with the commonest manifestation being peripheral ulcerative keratitis and scleritis. Only one patient had posterior segment involvement, specifically posterior ischemic optic neuropathy. Two patients demonstrated unilateral involvement. There was only one patient diagnosed with systemic involvement, presenting as mixed nephrotic and nephritic syndrome, who required a course of intravenous methylprednisolone during the active stage. Three patients required second-line immunosuppressants throughout the course due to frequent relapse.

Conclusion

Anterior segment ocular manifestations are not uncommon and can be the initial presentation of p-ANCA-associated vasculitis (AAV). Therefore, its evaluation should be considered in establishing the diagnosis of AAV, a life-threatening disease.

KEYWORDS:

• ANCA-associated vasculitis
• perinuclear antineutrophil cytoplasmic antibody
• peripheral ulcerative keratitis
• posterior ischemic optic neuropathy
• scleritis

Acknowledgments

The authors would like to thank the rheumatologist for the co-management of the patients and patients for the consent of sharing the clinical photos for publication.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.