Superior Limbic Keratoconjunctivitis-like Inflammation in Patients with Chronic Graft-Versus-Host Disease - PubMed (original) (raw)

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

Kavitha R Sivaraman et al. Ocul Surf. 2016 Jul.

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 BostonSight® 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.

Keywords: Limbal stem cell dysfunction; ocular chronic graft-versus-host disease; prosthetic replacement of the ocular surface (PROSE); superior limbic keratoconjunctivitis.

Copyright © 2016 Elsevier Inc. All rights reserved.

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Figures

Figure 1

Figure 1

Slit lamp photograph shows the fluorescein staining pattern of the left eye of Patient 5. Note the superior corneal staining indicative of SLK-like inflammation and limbal stem cell dysfunction. This is in addition to the band of interpalpebral dry-eye related staining in the inferior half of the cornea.

Figure 2

Figure 2

Right (A) and left (B) eyes of Patient 1 showing sectoral superior conjunctival injection consistent with SLK-like inflammtation. Treatment with scleral contact lenses led to significant improvement in the ocular surface and subjective symptoms.

Figure 3

Figure 3

Superior conjunctival injection and corresponding rose bengal staining in the left eye of patient 7.

Figure 4

Figure 4

Slit lamp photograph of the left (A) and right (B) eyes of Patient 4. The patient developed severe superior limbal stem cell dysfunction due to untreated superior limbic keratoconjunctivitis. This subsequently led to permanent limbal stem cell deficiency and corneal vascularization.

Figure 5

Figure 5

Slit lamp photographs with (A) and without (B) fluorescein staining of the left eye of Patient 10 showing frank superior limbal stem cell deficiency with superior corneal neovascularization and pannus. This patient was initially evaluated at our clinic 3 years earlier and was noted to have SLK-like inflammation in both eyes (left worse than right), with superior corneal staining but no neovascularization. Patient was subsequently lost to follow-up and returned with frank limbal stem cell deficiency in the left eye.

Figure 6

Figure 6

Summary of response to treatment of eyes with superior limbic keratoconjunctivitis-like inflammation and chronic graft-versus-host disease. SLK: superior limbic keratoconjunctivitis. cGVHD: chronic graft-versus-host disease.

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