Journal Article

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

Alfonso Eirin, Maria V. Irazabal, Morie A. Gertz, Angela Dispenzieri, Martha Q. Lacy, Shaji Kumar, Sanjeev Sethi, Samih H. Nasr, Lynn D. Cornell, Mary E. Fidler, Fernando C. Fervenza and Nelson Leung

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 27, issue 3, pages 1097-1101
Published in print March 2012 | ISSN: 0931-0509
Published online November 2011 | e-ISSN: 1460-2385 | DOI:
Clinical features of patients with immunoglobulin light chain amyloidosis (AL) with vascular-limited deposition in the kidney

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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 ± 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 V-AL, 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 λ 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

Journal Article.  2726 words.  Illustrated.

Subjects: Nephrology

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