A CASE STUDY OF EPITHELIAL OFF CORNEAL CROSSLINKING IN A PATIENT WITH RHEUMATOLOGICAL DISEASE

To report a successful treatment of keratoconus via epithelial off corneal crosslinking (epi-off CXL) in a patient with previously uncontrolled rheumatologic disease.

Private 2 provider ophthalmology practice located in Upstate New York, United States.

A 47 year old white male with a prior diagnosis of progressive KCN (OS>OD) was referred for epi-off CXL (epi-on not FDA approved in the United States). After a complete exam, he was scheduled for CXL. One week prior to his procedure it was recognized that the patient did not inform the physician of his previous diagnosis of ankylosing spondylitis. He had been previously treated with Humira (Adalimumab) but was currently off of it. In addition, he complained of extreme neck pain.  The procedure was subsequently cancelled and he was referred back to his rheumatologist. Bloodwork confirmed elevated inflammatory markers and he began Remicade (Infliximab) infusions.

After 3 months the patient’s neck pain was controlled and his inflammatory markers were found to be within normal limits. While he was cleared from a rheumatologic perspective, concerns remained for delayed epithelial healing / corneal melt. The patient was offered epi-on CXL with three other providers but refused due to cost. With only epi-off an option for the patient, a literature search to examine CXL with rheumatologic disease yielded no protocols. Therefore, we searched for corneal procedures (e.g. penetrating keratoplasty, clear corneal incisions) in high risk rheumatologic patients (chiefly, S.P. Chow, et al., 2015 [DOI:10.1097/ICO.0000000000000615] and Styliandies et al., 2013 [DOI: 10.1016/j.ophtha.2012.09.050]) as well as consulted with colleagues Dr. R, Shetty and Dr. F. Hafezi. These findings and suggestions were adopted into the patient’s care plan, including: 1) a 6.5 mm zone of epithelial removal done manually with EBK blade; the peripheral cornea was untouched; 2) assessing Sedimentation Rate, CRP, and vitamin D levels; 3) preoperative and post-operative treatment with topical cyclosporine and a 6 day taper of oral prednisone (in mg by day: 20, 20, 10, 10, 5, 5) beginning one day prior to CXL; 4) Post-operative treatment with topical prednisolone acetate for one month; 5) earlier follow-ups: 1 day, 4 day, and 1 week; and 6) timing his CXL between infusions. The patient subsequently had uneventful epi-off CXL.

This case study represents a successful epi-off CXL treatment of a previously rheumatologically uncontrolled patient. Quieting the patient’s rheumatologic condition, careful planning, co-management with rheumatology, as well as additional precautions for epi-off CXL afforded a contraindicated patient to receive treatment for KCN and avoid the need for a corneal transplant. We welcome further inputs and studies from our colleagues to assist in the creation of a formalized protocol to bring treatment to this previously contraindicated population.