Clinical Image: Scleral melt in granulomatosis with polyangiitis

The patient, a 26-year-old White male with a 4-year history of granulomatosis with polyangiitis (GPA), was evaluated for redness and a painless bulge in the right eye. Initial diagnosis of GPA included unilateral right-sided episcleritis, upper airway involvement, and pulmonary infiltrates. Nasal mucosa biopsy confirmed necrotizing granulomatous inflammation and laboratory assessment demonstrated positive antineutrophil cytoplasmic antibodies directed against proteinase 3. He was initially treated with glucocorticoids (GCs) and methotrexate (switched to azathioprine later because of side effects). At the time of presentation to ophthalmology, evaluation noted scleral inflammation in the superior quadrant associated with scleral thinning and protrusion of globe contents, consistent with scleral melt (arrow: globe protrusion). He did not have other features suggestive of active systemic vasculitis, and he had been off immunosuppressive therapy. The patient was started on remission induction therapy with rituximab and GCs but eventually required placement of multiple scleral patch grafts and amniotic membrane transplant surgeries. Scleritis and episcleritis are the most common ocular manifestations in GPA (1). Scleral involvement is a vision-threatening and severe manifestation of GPA (2, 3). Although remission of ocular involvement can be achieved in the majority of patients with scleritis with prompt recognition and treatment initiation, recalcitrant disease can result in scleral ischemia causing compromised scleral integrity requiring surgical intervention (1). Longitudinal follow-up for patients with GPA and ocular involvement is crucial, because this can be the only sign of GPA activity and can result in major comorbidity. Management of ocular involvement in GPA should be closely coordinated between rheumatology and ophthalmology. Disclosure form Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.