Relationship of ocular presentation in granulomatosis with polyangiitis to autoantibodies and disease activity

The aim of this study was to assess disease characteristics, autoantibodies, and disease activity in granulomatosis with polyangiitis (GPA) patients with ocular manifestations.

The cohort included 46 GPA patients visiting the ophthalmology clinic. Ocular manifestations were recorded, clinical and slit lamp examinations were performed. The Birmingham Vasculitis Activity Score (BVAS) was recorded. Laboratory investigations and the antineutrophil cytoplasmic antibody (ANCA) assay were performed.

Median age of the 22 male and 24 female patients was 44.5 (32-63) years, median disease duration 6.5 (1-16) years. Ocular manifestations were present in all patients: 12 (26.1%) had proptosis; 40 (87%) had scleritis/episcleritis, with perforation in 3 (6.5%); 33 (71.7%) had keratoconjunctivitis (KC), with acute infiltrative stromal keratitis in 11, peripheral ulcerative keratitis in 15, and sclerosing keratitis in 11 patients. Uveitis was present in 11 (23.9%) and retinal changes including vasculitis, exudates, and hemorrhage were present in 7 (15.2%). Blurred vision was present in 43 (93.5%) patients and 2 (4.3%) had vision loss. Glaucoma was present in 4 (8.7%) and hypotony in 2 (4.3%) patients. Involvement was bilateral in 32 (69.6%) patients. Rheumatoid factor (RF) was positive in 56.5% and significantly associated with uveitis (p = 0.04), while antinuclear antibody (ANA) was positive in 45.7% and significantly associated with KC (p = 0.04). BVAS tended to be higher in patients with uveitis (p = 0.49).

Ocular involvement must be considered in all GPA patients and referral to an experienced ophthalmologist is mandatory for proper management and improved outcome of such a rare systemic disease. ANA and RF positivity may raise suspicion for KC or uveitis, respectively. There was a remarkable association between uveitis and disease activity.