Use of cyclosporin in the treatment of steroid resistant post-keratoplasty atopic sclerokeratitis

Abstract

Aims-To demonstrate that oral cyclosporin is successful in the management of steroid refractory post-keratoplasty atopic sclerokeratitis (PKAS). Methods-Series of three patients.Results-The patients developed PKAS between 2 and 11 days after surgery. All cases failed to respond to oral prednisolone 60-80 mg daily for 10-30 days but resolved with the addition of systemic cyclosporin 5.0-7.5 mg/kg/day. There have been no relapses following withdrawal of therapy after 3-18 months. Conclusions-PKAS is an uncommon, but severe, complication of corneal graft surgery and often responds to oral prednisolone treatment. Oral cyclosporin results in rapid resolution of PKAS in steroid unresponsive cases. (Br J Ophthalmol 2001;85:91-92) Penetrating keratoplasty may be required to treat keratoconus and corneal scarring associated with atopy related eye diseases.1 Intense inflammation can occur shortly after surgery resulting in wound dehiscence, failure of epithelialisation, and early rejection called post-keratoplasty atopic sclerokeratitis (PKAS).2 This condition occurs uncommonly particularly in atopic individuals with high (>1000 IU/ml) serum IgE. They present up to 10 days postoperatively with severe photophobia, pain, epiphora, mucus discharge, intense conjunctival and anterior scleral injection with mucus adherent to the sutures which can cheesewire through the inflamed tissues leading to wound slippage and frank dehiscence. Later, microbial keratitis and graft rejection supervene. However, if treated early with high dose oral prednisolone, in many patients the inflammation can be controlled quickly and the prognosis is good with no recurrences. 2 We describe three cases in which oral steroids did not control the inflammation and cyclosporin was added with rapid improvement. Although therapy over many months was required, both drugs were eventually stopped. Case reports CASE 1This 25 year old man with previous vernal keratoconjunctivitis had keratoconus diagnosed at the age of 9. At surgery the best corrected left acuity was 6/12 and 6/120 right with a cone too steep for contact lens fitting. His serum IgE was 120 IU/l preoperatively. A right (8 mm) penetrating keratoplasty was performed using 16 interrupted sutures and subconjunctival betnesol at the end of surgery.The day after surgery there was minimal inflammation but at 4 days postoperatively, he developed acute inflammation, photophobia, and pain in the right eye and was diagnosed as having PKAS. Dexamethasone eye drops 0.1% were increased to hourly and prednisolone 80 mg daily was added. After 8 days (day 15 postoperatively), there was less pain but severe inflammation remained with anterior scleritis and infiltrates at the host insertion of some sutures. Oral cyclosporin (Sandimmun, Novartis), was added at 5 mg/kg/day. The inflammation completely resolved over the subsequent 12 days (by 27 days postoperatively) and the prednisolone was reduced over the following 3 months when it was stopped, followed by the cyclosporin. Two loose corneal sutures were...

The management of severe sclerokeratitis following corneal transplantation surgery

“…It has been noted that patients with additional risk factors for inflammation may be refractory to systemic corticosteroids alone. 9 There are now several newer agents available-rapamycin, tacrolimus, mycophenolate mofetil, and anti-tumour necrosis factor agents-that may have a role in the future. It should be noted that all these agents are associated with a potential element of additional morbidity.…”

Immunotherapy for corneal inflammatory disorders: stepping up and down the ladder

“…Figure 1 shows an algorithm for any non-pemphigoid-related inflammation, such as Mooren's ulcer, rheumatoid melt or Wegener's granulomatosis, atopic conjunctivitis or sclerokeratitis,1 Stevens Johnson syndrome with recurrent inflammation or scleritis,2 acanthamoeba scleritis,3 and necrotising scleritis.…”

“…Adding a short course of high-dose corticosteroids to any of these agents can help to control inflammation more rapidly. Ciclosporin and other calcineurin inhibitors work particularly well in atopic disease 1 4. Ciclosporin is well tolerated in patients younger than 60 years of age.…”

Hornhauteinschmelzung nach Cross-Linking und tiefer lamellärer Keratoplastik („DALK”) bei Keratokonus

“…Meistens erfolgt eine rasche Besserung nach Gabe von systemischen Steroiden. Falls diese nicht zum Erfolg führt, kann die Therapie mit Cyclosporin in einer Dosierung von 5,0 -7,5 mg/kg KG/d (talspiegeldosiert) ergänzt werden[1,4].…”