Peripheral Ulcerative Keratitis And Rheumatoid Arthritis: The Devastating Impact Of Medication Non-Adherence On Corneal Integrity

Peripheral ulcerative keratitis (PUK) is a rare but vision-threatening disorder characterized by crescentic stromal thinning and ulceration of the peripheral cornea. It is often associated with systemic autoimmune diseases particularly rheumatoid arthritis(RA), where immune-mediated inflammation causes stromal destruction. However, PUK may occur without previously diagnosed systemic disease, making diagnosis difficult. Due to rapid progression and risk of corneal melting or perforation, PUK is an ophthalmic emergency. We present a case of RA-associated PUK with severe peripheral corneal thinning requiring urgent surgical management and highlight the importance of early multidisciplinary treatment to prevent vision-threatening complications.

This case was managed in a tertiary ophthalmology referral center with slit-lamp biomicroscopy and anterior segment OCT (AS-OCT). Due to the high risk of corneal perforation, the patient was assessed urgently and carefully. A multidisciplinary approach involving Ophthalmology and Rheumatology teams was promptly established. Surgical treatment was performed in the operating room and postoperative follow-up included close anterior segment monitoring and detailed systemic inflammatory evaluation.

An 88-year-old male presented with a 10-day history of ocular pain, foreign body sensation, tearing, and redness in the left eye. Ophthalmic evaluation included best-corrected visual acuity (BCVA), intraocular pressure (IOP), slit-lamp and fundus exam and AS-OCT. Previous records were reviewed. Medical history showed long-standing RA with irregular follow-up and poor adherence. He used methotrexate, prednisolone, hydroxychloroquine, and leflunomide. The patient reported persistent joint pain and morning stiffness. Rheumatologic consultation and exam revealed pain, swelling, redness, and warmth in the right foot with rheumatoid hand deformities. Laboratory tests, including inflammatory markers and rheumatologic tests, were assessed.

At presentation, BCVA was counting fingers at 1 m in the right eye and hand motion in the left eye. IOP was 14/15 mmHg. Slit-lamp exam showed peripheral corneal thinning with epithelial defect and stromal infiltration consistent with peripheral ulcerative keratitis, conjunctival hyperemia, without perforation. Fundus exam showed a pale optic disc in the right eye and normal left fundus. AS-OCT showed stromal thinning to ~90 µm with central corneal thickness 570 µm, indicating perforation risk. RF and anti-CCP were positive. Multilayer amniotic membrane transplantation was performed. Postoperatively the ocular surface stabilized and corneal integrity remained intact. Rheumatology considered seropositive refractory RA and planned treatment.

Rheumatoid arthritis–related peripheral ulcerative keratitis is a vision-threatening manifestation of systemic vasculitis. Progressive stromal destruction reflects increased MMP activity and pro-inflammatory cytokines, particularly TNF-α. Early recognition by the ophthalmologist and prompt treatment are essential to prevent corneal melting and perforation. Amniotic membrane transplantation provides tectonic support, promotes epithelial healing, reduces inflammation, and stabilizes the ocular surface during the acute phase. Careful ophthalmic follow-up can be vision-saving for these patients. Close collaboration with rheumatology and early systemic therapy are key to controlling the disease and preventing irreversible visual loss.