Abstract
BACKGROUND: In this report, we present the results of a severe case of Sjögren's syndrome-related keratopathy after fluocinolone acetonide 190-μg intravitreal implant (Iluvien®; Alimera Sciences Inc.) therapy. CASE PRESENTATION: A 52-year-old Caucasian woman with Sjögren's syndrome secondary to autoimmune hepatitis and primary sclerosing cholangitis was admitted to our emergency department owing to bilateral corneal ulcers and corneal perforation in the left eye following exposure keratopathy in an artificially induced coma. Within the following months, recurrent fulminant keratolysis with perforations required multiple penetrating keratoplasties and amniotic membrane transplants in both eyes. With new signs of severe keratolysis, an intravitreal fluocinolone acetonide implant was injected off-label in the left eye, and a third penetrating keratoplasty was performed 2 weeks later. In the 6 months of follow-up after the last penetrating keratoplasty, no more surgical interventions were needed in the eye with the fluocinolone acetonide implant. The corneal surface remained stable, and intraocular pressure was normal. During this time frame, two further penetrating keratoplasties, one vitrectomy, and five amniotic membrane transplants were performed in the fellow eye owing to relapsing keratolysis and perforations. CONCLUSIONS: To the best of our knowledge, this is the first report of fluocinolone acetonide intravitreal therapy in a patient with corneal disease. In the 6-month follow-up period, no surgical intervention was needed in the eye with the fluocinolone acetonide implant, whereas further penetrating keratoplasties and amniotic membrane transplants were performed in the fellow eye. Intravitreal fluocinolone acetonide may be considered as a treatment option in severe cases of autoimmune corneal disease.