Recalcitrant sterile keratolysis of corneal graft associated with systemic flare-up of rheumatoid arthritis: A case report and review of literature.

PURPOSE: To report a rare case of recalcitrant sterile corneal graft melt following routine optical penetrating keratoplasty in a patient with rheumatoid arthritis (RA), and to highlight the role of systemic disease exacerbation and tumor necrosis factor-alpha (TNF-α) inhibition in stabilizing the condition.

METHODS: A 54-year-old female with well-controlled RA underwent an optical triple procedure for a visually significant central adherent leukoma. Postoperative follow-up revealed recurrent sterile keratolysis of the donor cornea coinciding with systemic flare-ups of RA. A standard microbiological and serological workup was performed to rule out infectious and other systemic causes.

RESULTS: Despite appropriate topical and systemic therapy, including corticosteroids and re-suturing with amniotic membrane transplant, the corneal melt persisted and recurred. All infectious causes were ruled out. A significant rise in inflammatory markers correlated with systemic RA activity. Initiation of adalimumab led to stabilization of the corneal melt within two weeks, along with marked improvement in systemic symptoms. At 6 months, the graft remained opaque but tectonically stable, and the globe's structural integrity was preserved. No recurrence was noted at 18-month follow-up.

DISCUSSION: Sterile corneal melt has been described in association with surgical interventions, drug toxicity, and systemic autoimmune diseases, particularly rheumatoid arthritis (RA), where dysregulated inflammatory pathways lead to keratolysis, typically presenting as peripheral ulcerative keratitis. In contrast, our case is unique in that keratolysis was confined predominantly to the donor corneal graft and graft-host junction following an otherwise uneventful optical penetrating keratoplasty. Infectious causes, NSAID toxicity, dry eye disease, and Sjögren syndrome were excluded. At the same time, the selective involvement of the donor tissue, negative microbiology, and elevated rheumatoid factor and inflammatory markers supported an immune-mediated graft melt related to rheumatic eye disease. Unlike in previously reported cases after lamellar or endothelial keratoplasty, in which melts originated in the host cornea or were associated with epithelial healing defects or Sjögren syndrome, our patient had complete initial graft epithelialization and normal ocular surface parameters. The temporal association of graft melt with a systemic RA flare, high RA factor titers, and involvement of the graft-host junction suggests a strong link between systemic inflammatory activity and graft survival. This case highlights a rare presentation of autoimmune-mediated sterile graft melt after routine penetrating keratoplasty. It underscores the importance of recognizing systemic disease activity as a critical factor in postoperative corneal outcomes.

CONCLUSION: This case highlights the potential for sterile graft melt to manifest as a systemic autoimmune flare in RA. Early recognition and multidisciplinary management, including biologic agents such as adalimumab, may be crucial for preserving ocular integrity in such recalcitrant cases.