Posters

Recurrent corneal melts in primary Sjögren’s syndrome

Poster Details

First Author: P.Scollo UK

Co Author(s):    M. Rana   S. Rauz                 

Abstract Details

Purpose:

Primary Sjögren’s Syndrome (pSS), considered an orphan disease due to unknown aetiology, is a chronic immune-mediated disorder involving lymphocytic infiltration of exocrine glands causing dry eyes and mouth. Corneal melting as an initial presentation in pSS is a rare but very serious complication that may require penetrating keratoplasty and immunosuppression. Currently, no evidence exists to indicate which combination of immunosuppression improves the five-year survival of high risk corneal transplants, which continue to have a poorer prognosis than liver transplants. We highlight the difficulties encountered in such a high risk case and the medical/surgical strategies adopted to tackle them.

Setting:

The Birmingham and Midland Eye Centre, United Kingdom.

Methods:

Case report with 3 year follow-up. Repeated bilateral perforating corneal melts that were central, indicating dry eye as the likely causative factor rather than peripheral ulcerative keratitis. Treatment involved multiple penetrating keratoplasties (six allografts required in total) with additional strategies involving amniotic membrane grafts, serum eye drops, medically and surgically induced tarsorraphies, subconjunctival and limbal avastin injections and numerous immunosuppressive treatments (each fraught with their own adverse effects) all in attempt to prolong survival of subsequent grafts following initial failures.

Results:

Recovery was further complicated by an episode of endophthalmitis which may have been endogenous secondary to immunosuppressive treatment or exogenous secondary to surgery or possibly further perforation – the latter being ruled out on examination. Diagnostic vitrectomy revealed a mixed fungal and bacterial cause. Later, a possible source of self-inoculation was revealed as the patient had suffered recent bouts of vaginal candidiasis, for which she was at greater risk due to her pSS and immunosuppression. Patient now stable with no further melts for 10 months, on systemic mycophenolate, topical dexamethasone and intensive lubrication. Photographs demonstrate corneal melts and current state.

Conclusions:

pSS may present with corneal melting. Until sufficient evidence for optimal immunosuppressive strategies to prolong corneal graft survival is elucidated we may be guided by renal transplant strategies which suggest a combinatory approach using mycophenolate (a myelosuppressive agent) and sirolimus (mTOR inhibitor) potentially having an edge over mycophenolate alone, or mycophenolate in combination with cicosporin. Regarding pSS, no studies have investigated immunosuppression looking at ocular outcomes making it difficult to know if systemic treatment alters sicca symptomatology. Thus, prolongation of graft survival strategies may not work for sicca, which in our case is possibly the primary cause of corneal melting.

Financial Disclosure:

NONE