Abstract
Purpose:
Vernal Keratoconjuncivitis (VKC) is a chronic inflammatory disorder, typically of young males. In severe cases it causes significant burning, itching and discharge; with corneal vascularization, shield ulcers and visual loss. Such cases are often challenging to manage with conventional therapies. We here describe the successful use of systemic ciclosporine in the management of refractory VKC in children.
Methods:
A prospective observational study of 5 children with severe refractory VKC (cobblestone papillae, corneal erosions and shield ulceration), who were commenced on systemic ciclosporine. Baseline and outcome data was collected on symptoms (burning, photophobia, discharge, itching and blurred vision), visual acuity, and clinical signs (injection, discharge, papillae, ptosis, trantas dots, corneal erosions, corneal vascularization and shield ulceration), together with ciclosporine serum levels and side effects.
Results:
5 boys (aged 6.25-13.75, mean 10.5 years) were commenced on systemic ciclosporine at 2.5mg/kg daily in divided doses. Ciclosporine was well tolerated in 4 cases. In 1 case, dosing was reduced to 2mg/kg daily and then discontinued due to deranged liver function tests. Of the 4 well tolerated cases: 2 cases achieved complete resolution within 3 months; 1 case achieved complete resolution at 6 months following a single recurrent exacerbation (burning and cobblestone papillae) which prompted increased dosing to 4mg/kg daily; 1 case remained to have persistent but reduced activity (cobblestone papillae and corneal epitheliopathy) at an increased dose of 4mg/kg daily, with frequent exacerbations in the first 12 months but completely resolved thereafter. Serum ciclosporine levels across all cases were 18-82µg/l, significantly below the therapeutic range for post-transplant immunosuppression.
Conclusions:
Systemic ciclosporine offers a novel, effective and usually well tolerated treatment for severe refractory VKC in children. It should be considered as an adjuvant therapy in such cases.
Keywords: 475 conjunctivitis •
489 cyclosporine •
555 immunomodulation/immunoregulation