title: Natural history and outcome of light chain deposition disease.
abstract: Light chain deposition disease (LCDD) is characterized by the deposition of monotypic immunoglobulin light chains in the kidney, resulting in renal dysfunction. Fifty-three patients with biopsy-proven LCDD were prospectively followed at the UK National Amyloidosis Center. Median age at diagnosis was 56 years, and patients were followed for a median of 6.2 years (range, 1.1-14.0 years). Median renal survival from diagnosis by Kaplan-Meier analysis was 5.4 years, and median estimated patient survival was 14.0 years; 64% of patients were alive at censor. Sixty-two percent of patients required dialysis, and median survival from commencement of dialysis was 5.2 years. There was a strong association between hematologic response to chemotherapy and renal outcome, with a mean improvement in glomerular filtration rate (GFR) of 6.1 mL/min/year among those achieving a complete or very good partial hematologic response (VGPR) with chemotherapy, most of whom remained dialysis independent, compared with a mean GFR loss of 6.5 mL/min/year among those achieving only a partial or no hematologic response (P < .009), most of whom developed end-stage renal disease (ESRD; P = .005). Seven patients received a renal transplant, and among those whose underlying clonal disorder was in sustained remission, there was no recurrence of LCDD up to 9.7 years later. This study highlights the need to diagnose and treat LCDD early and to target at least a hematologic VGPR with chemotherapy, even among patients with advanced renal dysfunction, to delay progression to ESRD and prevent recurrence of LCDD in the renal allografts of those who subsequently receive a kidney transplant.
authors.affiliation: National Amyloidosis Centre and Centre for Nephrology, Division of Medicine, University College London, London, United Kingdom.
authors.forename: Rabya H
authors.initials: RH
authors.lastname: Sayed
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Ashutosh D
authors.initials: AD
authors.lastname: Wechalekar
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Janet A
authors.initials: JA
authors.lastname: Gilbertson
authors.affiliation: Centre for Nephrology, Division of Medicine, University College London, London, United Kingdom.
authors.forename: Paul
authors.initials: P
authors.lastname: Bass
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Shameem
authors.initials: S
authors.lastname: Mahmood
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Sajitha
authors.initials: S
authors.lastname: Sachchithanantham
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Marianna
authors.initials: M
authors.lastname: Fontana
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Ketna
authors.initials: K
authors.lastname: Patel
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Carol J
authors.initials: CJ
authors.lastname: Whelan
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.affiliation: National Amyloidosis Centre and.
authors.forename: Julian D
authors.initials: JD
authors.lastname: Gillmore
mesh_terms: D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000401:mortality / Q000473:pathology* / Q000628:therapy
chemical_list: D007147:Immunoglobulin Light Chains