Ophthalmic manifestations and visual outcomes of granulomatosis with polyangiitis: a retrospective multicentre study in Korea.

OBJECTIVES

To analyse the ophthalmic manifestations and treatment outcomes of Korean patients with granulomatosis with polyangiitis (GPA).

METHODS

One hundred twenty patients diagnosed with GPA by rheumatologists from January 1984 to March 2019 at three referral centres were retrospectively reviewed. Patients with ophthalmic symptoms were examined by ophthalmologists and underwent orbital imaging. Ophthalmic manifestations were divided into ocular involvement and ocular adnexal involvement. Multivariable logistic regression was used to examine the factors related to ocular, ocular adnexal, and optic nerve involvement. Visual improvement was defined as a best-corrected visual acuity gain of ≥2 Snellen lines, accompanied by improvements in optic nerve function.

RESULTS

Ophthalmic manifestations were observed in 50 patients (41.7%) during the median follow-up period of 6.7 years. Proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) positivity (odds ratio 3.19, 95% confidence interval 1.18-8.60) was an independent risk factor for ocular involvement, while sinonasal involvement (21.94, 2.54-189.69) and brain involvement (5.38, 1.50-19.31) were independent risk factors for ocular adnexal involvement. Antinuclear antibody (ANA) positivity was associated with optic nerve involvement (12.8, 1.80-90.5). Visual improvement occurred in 5 of 14 patients with optic nerve involvement, all of whom received intravenous (IV) immunosuppressive treatments beyond oral steroids within 2 months of visual impairment.

CONCLUSIONS

Ophthalmic involvement is common in Korean GPA patients and should be considered in the presence of PR3-ANCA, sinonasal or brain involvement. Patients with positive ANA have an increased risk of optic nerve involvement, and early IV immunosuppressive treatments beyond oral steroids are necessary to improve the visual outcome.