A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation

PURPOSE: To report a case of Granulomatosis with Polyangiitis presenting with rare findings of cicatricial entropion progressing to significant symblepharon and fornix obliteration. OBSERVATIONS: An otherwise healthy patient with initial presentation findings of lower lid entropion with minimal cicatricial changes and a corneal ulcer. Management with an entropion Jones procedure repair produced good early postoperative results. 2 months post entropion repair, this patient represented with rapid progression to severe corneal ulceration, symblepharon formation and total obliteration of lower fornix and near complete obliteration of upper fornix of the left eye with concurrent acute kidney injury. He fulfilled diagnostic criteria for Granulomatosis with Polyangiitis (GPA). Systemic immunosuppressive treatment with prednisone and cyclophosphamide allowed acute kidney injury to recover however progressive cicatricial scarring ensued. CONCLUSIONS AND IMPORTANCE: Cicatricial entropion is a very rare presentation of GPA which can take years to progress however with surgical intervention, rapid recurrence and severe tarsal-conjunctival disease progression can ensue despite systemic immunosuppressive therapy. To prevent such unexpected surgical complication, we recommend a thorough systemic evaluation prior to consideration of lid surgery in any cases of cicatrising conjunctivitis presentation.