title: Long-term outcome of kidney transplantation in patients with fibrillary glomerulonephritis or monoclonal gammopathy with fibrillary deposits.
abstract: To determine the outcome of kidney transplantation in patients with fibrillary glomerulonephritis (FGN), a rare glomerular disease, we followed 12 patients, 5 with FGN and 7 patients with monoclonal gammopathy and fibrillary deposits (MGFD), who underwent 15 kidney transplants since 1988 with a median follow-up of 52 months. Recurrent disease did not arise in any of the patients with FGN but developed in 5 patients with MGFD. Seven allografts failed: 1 in the FGN group and 6 in the MGFD group. Median allograft survival for patients with MGFD was 37 months but had not been reached in FGN patients. One patient with FGN had primary allograft failure secondary to graft thromboembolism. Three patients with MGFD were re-transplanted and one lost the second allograft to recurrent disease, but the other two died from hematological malignancy. Another patient was diagnosed with MPGN type III and did not have detectable fibrillary material 22 months after transplantation. One patient with MGFD had stable allograft function 6 months post-transplant but another, with recurrent disease, underwent peripheral blood stem cell transplantation and regained stable allograft function. Our study shows that kidney transplantation appears safe in patients with FGN with little risk of recurrence. However, patients with MGFD have a significant risk for disease recurrence. Whether the development of hematological malignancies following transplantation in this group is related to their original disease or was coincidental requires further studies.
authors.affiliation: Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
authors.forename: Peter G
authors.initials: PG
authors.lastname: Czarnecki
authors.forename: Donna J
authors.initials: DJ
authors.lastname: Lager
authors.forename: Nelson
authors.initials: N
authors.lastname: Leung
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.forename: Fernando G
authors.initials: FG
authors.lastname: Cosio
authors.forename: Fernando C
authors.initials: FC
authors.lastname: Fervenza
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000401:mortality / Q000628:therapy*; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000401:mortality / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome