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 ± 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/1.73m(2), P = 0.01 and creatinine clearance 38.5 versus 64 mL/min/1.73m(2), 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.
authors: Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA.
authors: Alfonso
authors: A
authors: Eirin
authors: Maria V
authors: MV
authors: Irazabal
authors: Morie A
authors: MA
authors: Gertz
authors: Angela
authors: A
authors: Dispenzieri
authors: Martha Q
authors: MQ
authors: Lacy
authors: Shaji
authors: S
authors: Kumar
authors: Sanjeev
authors: S
authors: Sethi
authors: Samih H
authors: SH
authors: Nasr
authors: Lynn D
authors: LD
authors: Cornell
authors: Mary E
authors: ME
authors: Fidler
authors: Fernando C
authors: FC
authors: Fervenza
authors: Nelson
authors: N
authors: Leung
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000401:mortality / Q000503:physiopathology*; D016022:Case-Control Studies; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007668:Kidney / Q000503:physiopathology*; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D011507:Proteinuria / Q000209:etiology / Q000378:metabolism; D033581:Stem Cell Transplantation; D015996:Survival Rate; D014182:Transplantation, Autologous; D016896:Treatment Outcome; D014652:Vascular Diseases / Q000503:physiopathology*
chemical_list: D007147:Immunoglobulin Light Chains