Clinical features of patients with immunoglobulin light chain amyloidosis (AL) with vascular-limited deposition in the kidney

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Abstract

In the kidney, immunoglobulin light chain amyloidosis (AL) can be deposited in vascular-limited AL (V-AL) or diffuse (D-AL) pattern. These patterns are associated with different clinical presentations. A nested case study was performed to describe these differences. V-AL was defined by the vascular-limited deposits. Cases were matched for age, sex and date of renal biopsy. There were 12 cases of V-AL (mean age 61 6 11 years) and 24 cases of D-AL. Median follow-up was 26 months for V-AL and 38 months for D-AL, P = 0.14. Lambda was more common in D-AL (83.3%) than V-AL (50%, P = 0.04). Cardiac function was similar between the two groups. V-AL patients presented with lower renal function (serum creatinine = 2.1 versus 1.3 mg/dL, P = 0.02; estimated glomerular filtration rate 31 versus 59 mL/min/1.73m2, P = 0.01 and creatinine clearance 38.5 versus 64 mL/min/1.73m2, P = 0.02, respectively). Proteinuria was low grade in V-AL [0.4 (0.09-0.98) g/day] compared to nephrotic range in D-AL patients [8.0 (0.2-22) g/day, P < 0.001]. Stem cell transplantation was performed on 62.5% of the D-AL but on only 25% of the VAL, P = 0.08. Median survival was longer in patients with D-AL (77.2 months) versus V-AL (40.6 months, log-rank P = 0.02). Our study found that V-AL patients presented with more severe renal insufficiency and less proteinuria than D-AL. There was a preference for k light chain in the D-AL that was not noted in the V-AL. Patients with D-AL in this study had a longer median survival but most of them were stem cell transplantation candidates.

Original languageEnglish (US)
Pages (from-to)1097-1101
Number of pages5
JournalNephrology Dialysis Transplantation
Volume27
Issue number3
DOIs
StatePublished - May 2012

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Immunoglobulin Light Chains
Amyloidosis
Blood Vessels
Kidney
Stem Cell Transplantation
Proteinuria
Creatinine
Survival
Glomerular Filtration Rate
Renal Insufficiency

Keywords

  • AL
  • Amyloidosis
  • Proteinuria
  • Renal insufficiency
  • Vascular

ASJC Scopus subject areas

  • Nephrology
  • Transplantation

Cite this

@article{3c6f2badc5484cd395535803ac0248de,
title = "Clinical features of patients with immunoglobulin light chain amyloidosis (AL) with vascular-limited deposition in the kidney",
abstract = "In the kidney, immunoglobulin light chain amyloidosis (AL) can be deposited in vascular-limited AL (V-AL) or diffuse (D-AL) pattern. These patterns are associated with different clinical presentations. A nested case study was performed to describe these differences. V-AL was defined by the vascular-limited deposits. Cases were matched for age, sex and date of renal biopsy. There were 12 cases of V-AL (mean age 61 6 11 years) and 24 cases of D-AL. Median follow-up was 26 months for V-AL and 38 months for D-AL, P = 0.14. Lambda was more common in D-AL (83.3{\%}) than V-AL (50{\%}, P = 0.04). Cardiac function was similar between the two groups. V-AL patients presented with lower renal function (serum creatinine = 2.1 versus 1.3 mg/dL, P = 0.02; estimated glomerular filtration rate 31 versus 59 mL/min/1.73m2, P = 0.01 and creatinine clearance 38.5 versus 64 mL/min/1.73m2, P = 0.02, respectively). Proteinuria was low grade in V-AL [0.4 (0.09-0.98) g/day] compared to nephrotic range in D-AL patients [8.0 (0.2-22) g/day, P < 0.001]. Stem cell transplantation was performed on 62.5{\%} of the D-AL but on only 25{\%} of the VAL, P = 0.08. Median survival was longer in patients with D-AL (77.2 months) versus V-AL (40.6 months, log-rank P = 0.02). Our study found that V-AL patients presented with more severe renal insufficiency and less proteinuria than D-AL. There was a preference for k light chain in the D-AL that was not noted in the V-AL. Patients with D-AL in this study had a longer median survival but most of them were stem cell transplantation candidates.",
keywords = "AL, Amyloidosis, Proteinuria, Renal insufficiency, Vascular",
author = "Alfonso Eirin and {Irazabal Mira}, Maria and Morie Gertz and Angela Dispenzieri and Martha Lacy and Kumar, {Shaji K} and Sethi, {Sanjeev M} and Nasr, {Samih H.} and Cornell, {Lynn D.} and Fidler, {Mary E.} and Fervenza, {Fernando Custodio} and Nelson Leung",
year = "2012",
month = "5",
doi = "10.1093/ndt/gfr381",
language = "English (US)",
volume = "27",
pages = "1097--1101",
journal = "Nephrology Dialysis Transplantation",
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TY - JOUR

T1 - Clinical features of patients with immunoglobulin light chain amyloidosis (AL) with vascular-limited deposition in the kidney

AU - Eirin, Alfonso

AU - Irazabal Mira, Maria

AU - Gertz, Morie

AU - Dispenzieri, Angela

AU - Lacy, Martha

AU - Kumar, Shaji K

AU - Sethi, Sanjeev M

AU - Nasr, Samih H.

AU - Cornell, Lynn D.

AU - Fidler, Mary E.

AU - Fervenza, Fernando Custodio

AU - Leung, Nelson

PY - 2012/5

Y1 - 2012/5

N2 - In the kidney, immunoglobulin light chain amyloidosis (AL) can be deposited in vascular-limited AL (V-AL) or diffuse (D-AL) pattern. These patterns are associated with different clinical presentations. A nested case study was performed to describe these differences. V-AL was defined by the vascular-limited deposits. Cases were matched for age, sex and date of renal biopsy. There were 12 cases of V-AL (mean age 61 6 11 years) and 24 cases of D-AL. Median follow-up was 26 months for V-AL and 38 months for D-AL, P = 0.14. Lambda was more common in D-AL (83.3%) than V-AL (50%, P = 0.04). Cardiac function was similar between the two groups. V-AL patients presented with lower renal function (serum creatinine = 2.1 versus 1.3 mg/dL, P = 0.02; estimated glomerular filtration rate 31 versus 59 mL/min/1.73m2, P = 0.01 and creatinine clearance 38.5 versus 64 mL/min/1.73m2, P = 0.02, respectively). Proteinuria was low grade in V-AL [0.4 (0.09-0.98) g/day] compared to nephrotic range in D-AL patients [8.0 (0.2-22) g/day, P < 0.001]. Stem cell transplantation was performed on 62.5% of the D-AL but on only 25% of the VAL, P = 0.08. Median survival was longer in patients with D-AL (77.2 months) versus V-AL (40.6 months, log-rank P = 0.02). Our study found that V-AL patients presented with more severe renal insufficiency and less proteinuria than D-AL. There was a preference for k light chain in the D-AL that was not noted in the V-AL. Patients with D-AL in this study had a longer median survival but most of them were stem cell transplantation candidates.

AB - In the kidney, immunoglobulin light chain amyloidosis (AL) can be deposited in vascular-limited AL (V-AL) or diffuse (D-AL) pattern. These patterns are associated with different clinical presentations. A nested case study was performed to describe these differences. V-AL was defined by the vascular-limited deposits. Cases were matched for age, sex and date of renal biopsy. There were 12 cases of V-AL (mean age 61 6 11 years) and 24 cases of D-AL. Median follow-up was 26 months for V-AL and 38 months for D-AL, P = 0.14. Lambda was more common in D-AL (83.3%) than V-AL (50%, P = 0.04). Cardiac function was similar between the two groups. V-AL patients presented with lower renal function (serum creatinine = 2.1 versus 1.3 mg/dL, P = 0.02; estimated glomerular filtration rate 31 versus 59 mL/min/1.73m2, P = 0.01 and creatinine clearance 38.5 versus 64 mL/min/1.73m2, P = 0.02, respectively). Proteinuria was low grade in V-AL [0.4 (0.09-0.98) g/day] compared to nephrotic range in D-AL patients [8.0 (0.2-22) g/day, P < 0.001]. Stem cell transplantation was performed on 62.5% of the D-AL but on only 25% of the VAL, P = 0.08. Median survival was longer in patients with D-AL (77.2 months) versus V-AL (40.6 months, log-rank P = 0.02). Our study found that V-AL patients presented with more severe renal insufficiency and less proteinuria than D-AL. There was a preference for k light chain in the D-AL that was not noted in the V-AL. Patients with D-AL in this study had a longer median survival but most of them were stem cell transplantation candidates.

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KW - Amyloidosis

KW - Proteinuria

KW - Renal insufficiency

KW - Vascular

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U2 - 10.1093/ndt/gfr381

DO - 10.1093/ndt/gfr381

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