Juvenile Idiopathic Arthritis-Associated Peripheral Ulcerative Keratitis and Anterior Uveitis in a Pediatric Patient with Trisomy 21.

PURPOSE: Peripheral ulcerative keratitis (PUK) is a rare, vision-threatening disorder that typically occurs in adults with underlying autoimmune disease or infection. Few cases of pediatric PUK have been reported, making clinical guidelines rare.

METHODS

Case report.

RESULTS

A 13-year-old female with Trisomy 21 and well-controlled Graves' Disease and no history of ocular trauma presented with ten days of right eye pain refractory to treatment with topical antibiotics and corticosteroids. Visual acuity was count fingers and slit lamp examination showed marked conjunctival injection, as well as a fibrin band in the anterior chamber. Corneal examination demonstrated substantial inferior peripheral stromal thinning and overlying epithelial defect concerning for PUK, prompting hospital admission for management and systemic disease workup. Effusive asymmetric arthritis of the left lower ankle, left knee, and left hand was suspicious for juvenile idiopathic arthritis (JIA) and laboratory studies showed elevated CRP and ESR levels. Despite medical treatment with IV methylprednisolone 1000 mg daily and fortified antibiotic eye drops four times per day, a corneal descemetocele formed, prompting urgent surgical intervention. The patient underwent two interventions with amniotic membrane transplantation. Four months after initial presentation, visual acuity in the affected eye had improved to 20/25, same as fellow eye, and slit lamp examination showed corneal re-epithelialization, healed descemetocele, and ocular inflammatory control on methotrexate monotherapy. The final ocular diagnosis was PUK and anterior uveitis secondary to JIA.

CONCLUSION

Based on our clinical course, we recommend early systemic treatment to manage rapidly progressive corneal thinning in pediatric PUK. Early surgical intervention can be considered to avoid perforation.