Presumed Autoimmune Keratitis in Both Eyes Without Systemic Manifestations: A 40-Year Course of a Patient With Corneal Infiltrates and Melt

Peripheral corneal infiltration, corneal ulcer, and melt are recognized complications linked to systemic immunological diseases, such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. These manifestations, which occur in isolation, might be autoimmune keratitis but are difficult to prove underlying immunological abnormalities. This report described a patient with presumed autoimmune keratitis who repeatedly presented corneal infiltration and perforation in both eyes even after penetrating keratoplasty. The 68-year-old patient with a stable condition of keratoconjunctivitis sicca, in a 28-year follow-up, abruptly developed mild infiltrates in the corneal center of the right eye and white dense infiltrates in the peripheral and central cornea of the left eye. He was treated with topical 0.1% betamethasone eye drops and oral prednisolone tapering from 30 mg daily. The patient underwent cataract surgeries in both eyes 10 months after the onset of corneal infiltration and subsequently underwent penetrating keratoplasty in both eyes due to abrupt corneal perforation in the left eye 14 months after the onset of corneal infiltration. Six months post-keratoplasty, he experienced a recurrence of infiltrates in the corneal grafts in both eyes, leading to corneal leukoma in the left eye. The corneal graft in the right eye maintained its integrity with relatively mild opacity until approximately 3.5 years post-keratoplasty, when he abruptly developed white dense infiltration of both the corneal graft and his own peripheral cornea at the age of 73. In response to oral prednisolone tapered from 15 mg daily, the corneal infiltration in the right eye resolved but resulted in graft failure. Since he did not exhibit systemic symptoms and signs throughout the course, the repeat episodes of infiltration in both his own cornea and the corneal graft would be the manifestations of autoimmune keratitis. The entity of autoimmune keratitis in isolation would be beneficial to establish a therapeutic strategy for long-term immunosuppression in light of a risk for steroid side effects and a high rate of corneal graft failure.