Managing High-Risk Penetrating Keratoplasty In A Patient With Tectonic Graft Rejection History And Severe Sjögren's Syndrome.

The surgical management of corneal affection in severe Sjögren's syndrome poses a challenge for anterior segment surgeons due to the high complexity of corneal transplantation. These patients experience higher rates of rejection, often requiring numerous regrafts over the years. While advances in immunosuppressive therapy have improved systemic disease management, leading to decreased corneal complications, the distinct characteristics of these patients, require the implementation of high-risk keratoplasty protocols across all stages: preoperative, intraoperative, and postoperative. 

Son Llàtzer University Hospital.

We present a 76-year-old patient with severe Sjögren's syndrome for over 30 years. She has a history of multiple severe episodes of peripheral ulcerative keratitis, resulting in a leukomatous cornea with abundant vascularization and poor vision in her only functional right eye (left prosthetic eye). Systemically, she required flebogamma infusions, rituximab cycles, and chronic oral cyclosporine to control ocular flares. After years of ocular stability with systemic treatment combined with medroxyprogesterone, autologous serum, artificial tears and topical corticosteroids, she developed a neurotrophic ulcer with an inferior descemetocele, microperforation, and pancorneal neovascularization. Given the severity of the situation, emergency tectonic keratoplasty was performed, with the intention of implanting a Boston keratoprosthesis at a later stage. However, four months later, signs of rejection emerged, leading to the application of high-risk keratoplasty protocols, both topically and systemically: immunoglobulin infusions, rituximab, high-dose steroids (three megadoses), doxycycline, and 0.1% tacrolimus eye drops. Additionally, amniotic membrane transplantation with intrastromal bevacizumab injection was performed. Consequently, rejection was controlled, allowing for subsequent penetrating keratoplasty with 50% temporary tarsorrhaphy. One year later, the patient remains stable, having regained her baseline visual acuity, and prosthokeratoplasty has not been necessary. 

Patients with severe Sjögren's syndrome and recurrent ulcers require both medical and surgical optimization of the ocular surface before keratoplasty, as the corneal recipient bed is often highly vascularized, substantially increasing the risk of rejection. Considering this, amniotic membrane transplantation and intrastromal bevacizumab injections could prove beneficial in improving recipient bed conditions. The use of topical tacrolimus, along with systemic immunosuppression using bevacizumab, rituximab, and immunoglobulins during acute episodes, may be necessary. Additionally, partial tarsorrhaphy of 50% could be a therapeutic option for these patients.