PMID- 14655186
OWN - NLM
STAT- MEDLINE
DCOM- 20040422
LR  - 20220419
IS  - 1523-6838 (Electronic)
IS  - 0272-6386 (Linking)
VI  - 42
IP  - 6
DP  - 2003 Dec
TI  - Light chain deposition disease with renal involvement: clinical characteristics 
      and prognostic factors.
PG  - 1154-63
AB  - BACKGROUND: Light chain deposition disease (LCDD) is characterized by the tissue 
      deposition of monotypical immunoglobulin light chains (LCs). The aim of this 
      study was to investigate its clinical characteristics and prognostic factors. 
      METHODS: Multicenter study of LCDD with renal and patient survival analyses. 
      RESULTS: Sixty-three cases were studied (age: 58 +/- 14.2; males: 63.5%; 
      kappa/lambda deposition: 68/32%; underlying disorders: multiple myeloma [MM] 65%, 
      lymphoproliferative disorders 3%, idiopathic 32%). Ninety-six percent presented 
      with renal insufficiency (acute, 52%; chronic, 44%), and 84% with proteinuria >1 
      g/d. During the follow-up, 36 patients reached uremia (incidence rate: 23.7/100 
      patient-years) and 37 died (17.5/100 patient-years). The factors independently 
      associated with a worse renal prognosis were age (relative risk [RR], 1.05; 95% 
      confidence interval [CI], 1.009 to 1.086) and serum creatinine at presentation 
      (RR, 1.24; 95% CI, 1.02 to 1.5). Those independently associated with a worse 
      patient survival were age (RR, 1.06; 95% CI, 1.03 to 1.1), MM (RR, 2.75; 95% CI, 
      1.22 to 6.2), and extrarenal LC deposition (RR, 2.24; 95% CI, 1.15 to 4.35). 
      While kappa-LC deposition was more frequently associated with nodular sclerosing 
      glomerulopathy, histological parameters were not predictors of renal/patient 
      prognosis. The survival of the uremic patients undergoing dialysis was similar to 
      that of patients not reaching uremia. CONCLUSION: LCDD is characterized by renal 
      insufficiency with proteinuria and has a severe prognosis. Apart from age, the 
      prognostic factors identified were degree of renal insufficiency at presentation 
      affecting the renal prognosis, underlying hematologic disorder and extrarenal LC 
      deposition affecting the patient prognosis. Dialysis is worth performing in 
      uremic LCDD patients.
FAU - Pozzi, Claudio
AU  - Pozzi C
AD  - Department of Nephrology and Dialysis, A. Manzoni Hospital, Lecco, Italy. 
      c.pozzi@ospedale.lecco.it
FAU - D'Amico, Marco
AU  - D'Amico M
FAU - Fogazzi, Giovanni B
AU  - Fogazzi GB
FAU - Curioni, Simona
AU  - Curioni S
FAU - Ferrario, Franco
AU  - Ferrario F
FAU - Pasquali, Sonia
AU  - Pasquali S
FAU - Quattrocchio, Giacomo
AU  - Quattrocchio G
FAU - Rollino, Cristiana
AU  - Rollino C
FAU - Segagni, Siro
AU  - Segagni S
FAU - Locatelli, Francesco
AU  - Locatelli F
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PT  - Review
PL  - United States
TA  - Am J Kidney Dis
JT  - American journal of kidney diseases : the official journal of the National Kidney 
      Foundation
JID - 8110075
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Alkylating Agents)
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Immunoglobulin kappa-Chains)
RN  - 0 (Immunoglobulin lambda-Chains)
RN  - 0 (Immunosuppressive Agents)
RN  - AYI8EX34EU (Creatinine)
SB  - IM
MH  - Adrenal Cortex Hormones/therapeutic use
MH  - Adult
MH  - Age Factors
MH  - Aged
MH  - Aged, 80 and over
MH  - Alkylating Agents/therapeutic use
MH  - Creatinine/blood
MH  - Female
MH  - Humans
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Immunoglobulin kappa-Chains/metabolism
MH  - Immunoglobulin lambda-Chains/metabolism
MH  - Immunosuppressive Agents/therapeutic use
MH  - Kidney Diseases/*epidemiology/metabolism/pathology
MH  - Kidney Failure, Chronic/etiology/mortality
MH  - Life Tables
MH  - Male
MH  - Middle Aged
MH  - Multiple Myeloma/complications/epidemiology/metabolism/pathology
MH  - Paraproteinemias/complications/*epidemiology/metabolism/pathology/therapy
MH  - Plasmapheresis
MH  - Prognosis
MH  - Proportional Hazards Models
MH  - Retrospective Studies
MH  - Risk
MH  - Survival Analysis
MH  - Uremia/etiology/mortality
RF  - 25
EDAT- 2003/12/05 05:00
MHDA- 2004/04/23 05:00
CRDT- 2003/12/05 05:00
PHST- 2003/12/05 05:00 [pubmed]
PHST- 2004/04/23 05:00 [medline]
PHST- 2003/12/05 05:00 [entrez]
AID - S0272638603011685 [pii]
AID - 10.1053/j.ajkd.2003.08.040 [doi]
PST - ppublish
SO  - Am J Kidney Dis. 2003 Dec;42(6):1154-63. doi: 10.1053/j.ajkd.2003.08.040.