Clinical features of different orbital manifestations of granulomatosis with polyangiitis

The aim of this study was to distinguish different forms of orbital involvement in granulomatosis with polyangiitis (GPA) and to compare their clinical course and outcomes.

Two hundred twenty-six consecutive patients with GPA were retrospectively studied. All patients fulfilled the classification criteria of American College of Rheumatology; diagnosis of localized GPA was established according to Chapel Hill Consensus. Of those, 74 patients with orbital disease were enrolled into further investigation of different forms of orbital disease.

Overall ocular involvement was diagnosed in 50% (113/226) of patients; in 5.3% (12/226), inflammatory eye disease resulted in permanent visual loss. Most common ophthalmic manifestations were orbital masses, episcleritis/conjunctivitis, and scleritis (74/226, 32/226, and 12/226, respectively). Seventy-four patients with orbital involvement were divided into three groups: those with orbital mass without primary lacrimal gland involvement (1st group, 45 patients), those with lacrimal gland involvement (2nd group, 26 patients), and those with extraocular myositis (3rd group, 3 patients). Symptoms such as orbital pain, decreased vision, double vision, red eye, scleritis, orbital wall destruction, primary gaze strabismus, and ocular motility restriction were significantly more common in the 1st group. Peripheral ulcerative keratitis (PUK) was present in the 1st group only. Patients of the 1st group suffered from visual loss, double vision, primary gaze strabismus, and ocular motility restriction significantly more often. Eleven of them developed visual loss; 5 of them underwent enucleation, whereas in the group of patients with dacryoadenitis, only 1 patient had BCVA less than 0.3.

Three types of orbital disease in GPA were proposed: orbital mass without primary lacrimal gland involvement, dacryoadenitis, and extraocular myositis. Patients with orbital mass without lacrimal gland involvement are characterized with a higher rate of systemic disease, severe clinical course not uncommonly associated with other ophthalmic manifestations (necrotizing scleritis, PUK, orbital walls destruction), relatively unfavorable outcome with high level of morbidity (optic nerve atrophy, anophthalmos, strabismus), and higher rate of recurrences. Dacryoadenitis in GPA showed to be not severe manifestation with favorable prognosis.