Superior Limbic Keratoconjunctivitis-like Inflammation in Patients with Chronic Graft-Versus-Host Disease

Abstract Purpose Describe the presentation and management of superior limbic keratoconjunctivitis (SLK-like inflammation and secondary limbal stem cell dysfunction in the setting of ocular chronic graft-versus-host disease (cGVHD). Methods Retrospective observational case series in a multicenter cli...

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Published in:The ocular surface 2016-07, Vol.14 (3), p.393-400
Main Authors: Sivaraman, Kavitha R., MD, Jivrajka, Renu V., MD, Soin, Ketki, MD, Bouchard, Charles S., MD, Movahedan, Asadolah, MD, Shorter, Ellen, OD, Jain, Sandeep, MD, Jacobs, Deborah S., MD, Djalilian, Ali R., MD
Format: Article
Language:English
Subjects:
Chronic Disease
Corneal Diseases
Graft vs Host Disease
Humans
Inflammation
Keratoconjunctivitis
Limbal stem cell dysfunction
Limbus Corneae
ocular chronic graft-versus-host disease
Ophthalmology
prosthetic replacement of the ocular surface (PROSE)
Retrospective Studies
superior limbic keratoconjunctivitis
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Summary:Abstract Purpose Describe the presentation and management of superior limbic keratoconjunctivitis (SLK-like inflammation and secondary limbal stem cell dysfunction in the setting of ocular chronic graft-versus-host disease (cGVHD). Methods Retrospective observational case series in a multicenter clinical practice. Participants were 13 patients (26 eyes) with ocular cGVHD and SLK-like inflammation presenting to the University of Illinois at Chicago and Boston Foundation for Sight between January 1, 2009 and July 1, 2013. Main outcome measures 1) Reversal or worsening of SLK, and 2) development of limbal stem cell dysfunction. Results All eyes showed evidence of SLK-like inflammation and superior limbal stem cell dysfunction manifested by conjunctival injection and superior conjunctival and corneal staining. In addition to aggressive lubrication, management strategies for SLK included topical steroids (20/26), punctal occlusion (18/26), topical cyclosporine (24/26), autologous serum tears (12/26), therapeutic soft contact lens (13/26 eyes) and scleral lenses (4/26 eyes). SLK and limbal stem cell dysfunction were reversed in 23/26 eyes. Three eyes of two patients with long-standing disease demonstrated frank limbal stem cell deficiency (LSCD) and corneal pannus, with one patient requiring multiple reconstructive surgical procedures. Conclusions SLK-like inflammation is an under-recognized condition in patients with severe dry eyes secondary to ocular cGVHD. Untreated SLK can potentially lead to permanent LSCD over time. Early recognition and management of SLK in ocular cGVHD can improve vision, reverse signs, and may prevent these long-term consequences.
ISSN:1542-0124
1937-5913
DOI:10.1016/j.jtos.2016.04.003