PURPOSE

This study analyzed outcomes of orbital inflammation in granulomatosis with polyangiitis (GPA) and identified risk factors for unfavorable outcomes.

MATERIAL AND METHODS

The study included 107 patients with orbital GPA. A systemic form of the disease was identified in 37 (34.6%) patients, while a localized form of GPA was observed in 70 (65.4%) patients.

RESULTS

Visual acuity of 0.7 or higher was recorded in 69 (64.5%) patients, visual acuity below 0.7 was noted in 30 (28%) patients, blindness was detected in 3 (2.8%) cases, and anophthalmos in 5 (4.7%) patients. Oculomotor disorders were observed in the outcome in 44 (41.1%) patients. In 25 (23.4%) individuals, ocular motility was limited in one or more directions, with or without diplopia in those gaze positions, but in primary gaze there were no eye deviation or diplopia. Severe oculomotor impairment was characterized by the presence of strabismus and/or binocular diplopia in primary gaze position and was identified in 19 (17.7%) patients of the study gorup. Unfavorable prognostic factors included positive antineutrophil cytoplasmic antibodies (ANCA), a systemic form of the disease, diffuse orbital inflammation, and the presence of optic neuropathy and peripheral ulcerative keratitis (PUK) during the active phase of orbital inflammation.

CONCLUSION

In a large clinical cohort (107 patients), a favorable outcome of orbital inflammation in GPA was observed in 62.6% of cases, whereas an unfavorable outcome occurred in 27.4% of patients. Risk factors of an unfavorable outcome were identified for the first time, and a formula for predicting clinical outcome was proposed.