Long-term prognosis of AL and AA renal amyloidosis: a Japanese single-center experience.
BACKGROUND
Few studies have been conducted on the long-term prognosis of patients with amyloid light chain (AL) and amyloid A (AA) renal amyloidosis in the same cohort.


METHODS
We retrospectively examined 68 patients with biopsy-proven renal amyloidosis (38 AL and 30 AA). Clinicopathological findings at the diagnosis and follow-up data were evaluated in each patient. We analyzed the relationship between clinicopathological parameters and survival data.


RESULTS
Significant differences were observed in several clinicopathological features, such as proteinuria levels, between the AL and AA groups. Among all patients, 84.2 % of the AL group and 93.3 % of the AA group received treatments for the underlying diseases of amyloidosis. During the follow-up period (median 18 months in AL and 61 months in AA), 36.8 % of the AL group and 36.7 % of the AA group developed end-stage renal failure requiring dialysis, while 71.1 % of the AL group and 56.7 % of the AA group died. Patient and renal survivals were significantly longer in the AA group than in the AL group. eGFR of >60 mL/min/1.73 m2at biopsy and an early histological stage of glomerular amyloid deposition were identified as low-risk factors. A multivariate analysis showed that cardiac amyloidosis and steroid therapy significantly influenced patient and renal survivals.


CONCLUSIONS
Our results showed that heart involvement was the major predictor of poor outcomes in renal amyloidosis, and that the prognosis of AA renal amyloidosis was markedly better than that in previously reported cohorts. Therapeutic advances in inflammatory diseases are expected to improve the prognosis of AA amyloidosis.
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.|Department of Nephrology, Akita Red Cross Hospital, Akita, Japan.
Masatoyo
M
Ozawa
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan. komatsud@med.akita-u.ac.jp.
Atsushi
A
Komatsuda
Department of Nephrology, Akita Kousei Medical Center, Akita, Japan.
Hiroshi
H
Ohtani
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.
Mizuho
M
Nara
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.|Department of Nephrology, Akita Red Cross Hospital, Akita, Japan.
Ryuta
R
Sato
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.
Masaru
M
Togashi
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.
Naoto
N
Takahashi
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.|Department of Life Science, Akita University Graduate School of Engineering Science, Akita, Japan.
Hideki
H
Wakui
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000175:diagnosis / Q000276:immunology / Q000401:mortality / Q000628:therapy*; D001706:Biopsy; D009202:Cardiomyopathies / Q000276:immunology / Q000401:mortality / Q000628:therapy; D018450:Disease Progression; D005260:Female; D005355:Fibrosis; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology*; D007564:Japan; D053208:Kaplan-Meier Estimate; D007668:Kidney / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D007674:Kidney Diseases / Q000276:immunology / Q000401:mortality / Q000628:therapy*; D007676:Kidney Failure, Chronic / Q000276:immunology / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D011507:Proteinuria / Q000276:immunology / Q000401:mortality / Q000628:therapy; D006435:Renal Dialysis; D012189:Retrospective Studies; D012307:Risk Factors; D000685:Serum Amyloid A Protein / Q000276:immunology*; D013997:Time Factors; D016896:Treatment Outcome
D007147:Immunoglobulin Light Chains; D000685:Serum Amyloid A Protein
Amyloid A; Amyloid light chain; Japanese single-center cohort; Long-term prognosis; Renal amyloidosis