title: Intravenous immunoglobulin for antibody-mediated keratolimbal allograft rejection.
abstract: A 33-year-old woman with congenital aniridia presented with decreased vision in her right eye. Slit lamp examination revealed diffuse conjunctivalisation of the ocular surface with mild subepithelial fibrosis consistent with aniridic keratopathy secondary to limbal stem cell deficiency. She underwent limbal stem cell transplantation with cadaver donor tissue (keratolimbal allograft (KLAL) surgery) and received systemic immunosuppression. Despite optimal combination immunosuppressive therapy managed by a renal transplant specialist, 2 weeks after the KLAL, the patient developed intractable eye pain, conjunctival injection, dilation of the KLAL graft blood vessels and limbal haemorrhages. There were no epithelial defects noted. Donor-specific antibody testing was positive, and intravenous immunoglobulin therapy was initiated. There was immediate symptomatic and objective improvement. Fifteen months postoperatively, the patient's vision was 20/400 with a stable corneal epithelium and no evidence of inflammation.
authors.affiliation: McMaster University, Hamilton, Canada.
authors.forename: Victoria
authors.initials: V
authors.lastname: Squissato
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Canada.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Schiff
authors.affiliation: Department of Ophthalmology, University of Toronto, Toronto, Canada.
authors.forename: Clara C
authors.initials: CC
authors.lastname: Chan
mesh_terms: D000328:Adult; D064591:Allografts; D015783:Aniridia / Q000150:complications / Q000473:pathology; D000906:Antibodies; D003316:Corneal Diseases / Q000209:etiology / Q000601:surgery*; D016039:Corneal Transplantation / Q000009:adverse effects* / Q000379:methods; D004847:Epithelial Cells; D019573:Epithelium, Corneal / Q000166:cytology / Q000473:pathology*; D005260:Female; D006084:Graft Rejection / Q000188:drug therapy* / Q000517:prevention & control; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000009:adverse effects; D016850:Limbus Corneae / Q000166:cytology / Q000473:pathology; D011183:Postoperative Complications / Q000209:etiology / Q000601:surgery; D033581:Stem Cell Transplantation / Q000009:adverse effects; D013234:Stem Cells / Q000473:pathology; D014184:Transplantation, Homologous
chemical_list: D000906:Antibodies; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents