New trends in AA amyloidosis with renal involvement: a single-center experience

Abstract Introduction: In the northwest of Portugal, AA amyloidosis was reported as the most frequent amyloid nephropathy, but it is unclear if the disease’s incidence and outcomes have changed. The authors studied the changing epidemiology, aetiologies, and outcomes of patients with renal AA amylo...

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Main Authors: Nídia Marques, Catarina Oliveira-Silva, Ana Pinho, Ana Teresa Nunes, Susana Sampaio, Ana Oliveira, Isabel Tavares
Format: Article
Language:English
Published: Sociedade Brasileira de Nefrologia 2025-02-01
Series:Brazilian Journal of Nephrology
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002025000200302&lng=en&tlng=en
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author Nídia Marques
Catarina Oliveira-Silva
Ana Pinho
Ana Teresa Nunes
Susana Sampaio
Ana Oliveira
Isabel Tavares
author_facet Nídia Marques
Catarina Oliveira-Silva
Ana Pinho
Ana Teresa Nunes
Susana Sampaio
Ana Oliveira
Isabel Tavares
author_sort Nídia Marques
collection DOAJ
description Abstract Introduction: In the northwest of Portugal, AA amyloidosis was reported as the most frequent amyloid nephropathy, but it is unclear if the disease’s incidence and outcomes have changed. The authors studied the changing epidemiology, aetiologies, and outcomes of patients with renal AA amyloidosis over the last 40 years. Methods: This is a retrospective single-center cohort study involving patients with renal biopsy-proven AA amyloidosis diagnosed in the northwest of Portugal between 1978 and 2019. The patients were grouped into 14-year cohorts based on the year of diagnosis (CA 1978–1991; CB 1992–2005; CC 2006–2019), and clinical course and outcomes were analyzed. Results: Sixty-nine AA amyloidosis patients were included. The incidence of the disease remained stable in CA (64%) and CB (62.7%) as opposed to the significant decrease in the most recent cohort (44%, p = 0.027). The mean age at presentation increased by ten years from CA to CC. Overall, infections were the leading cause of death, with a significant rise over time (9.1% in CA to 76.9% at CC, p = 0.002). There were no significant global and renal survival differences between the three cohorts. However, the CC patients died at an older age (61.4 years) than the CA (52.3 years). Conclusion: The incidence of AA amyloidosis has been declining over the last 40 years. In contrast, the age at presentation of amyloid nephropathy has been increasing. Global and renal outcomes did not improve, but the average life expectancy increased, suggesting progress in general management and supportive care of renal and underlying pathology.
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spelling doaj-art-88d4deba9b754e519fec5ce6012e3e4a2025-02-04T07:41:22ZengSociedade Brasileira de NefrologiaBrazilian Journal of Nephrology2175-82392025-02-0147210.1590/2175-8239-jbn-2024-0127enNew trends in AA amyloidosis with renal involvement: a single-center experienceNídia Marqueshttps://orcid.org/0000-0001-5520-6899Catarina Oliveira-Silvahttps://orcid.org/0000-0002-5447-0644Ana Pinhohttps://orcid.org/0000-0002-9115-4683Ana Teresa Nuneshttps://orcid.org/0000-0002-3483-4981Susana Sampaiohttps://orcid.org/0000-0001-8626-9235Ana Oliveirahttps://orcid.org/0000-0002-5728-4572Isabel Tavareshttps://orcid.org/0000-0002-3497-8416Abstract Introduction: In the northwest of Portugal, AA amyloidosis was reported as the most frequent amyloid nephropathy, but it is unclear if the disease’s incidence and outcomes have changed. The authors studied the changing epidemiology, aetiologies, and outcomes of patients with renal AA amyloidosis over the last 40 years. Methods: This is a retrospective single-center cohort study involving patients with renal biopsy-proven AA amyloidosis diagnosed in the northwest of Portugal between 1978 and 2019. The patients were grouped into 14-year cohorts based on the year of diagnosis (CA 1978–1991; CB 1992–2005; CC 2006–2019), and clinical course and outcomes were analyzed. Results: Sixty-nine AA amyloidosis patients were included. The incidence of the disease remained stable in CA (64%) and CB (62.7%) as opposed to the significant decrease in the most recent cohort (44%, p = 0.027). The mean age at presentation increased by ten years from CA to CC. Overall, infections were the leading cause of death, with a significant rise over time (9.1% in CA to 76.9% at CC, p = 0.002). There were no significant global and renal survival differences between the three cohorts. However, the CC patients died at an older age (61.4 years) than the CA (52.3 years). Conclusion: The incidence of AA amyloidosis has been declining over the last 40 years. In contrast, the age at presentation of amyloid nephropathy has been increasing. Global and renal outcomes did not improve, but the average life expectancy increased, suggesting progress in general management and supportive care of renal and underlying pathology.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002025000200302&lng=en&tlng=enAmyloid Protein AAAmyloidosisEpidemiologyInflammation MediatorsChronic InfectionChronic Kidney Disease
spellingShingle Nídia Marques
Catarina Oliveira-Silva
Ana Pinho
Ana Teresa Nunes
Susana Sampaio
Ana Oliveira
Isabel Tavares
New trends in AA amyloidosis with renal involvement: a single-center experience
Brazilian Journal of Nephrology
Amyloid Protein AA
Amyloidosis
Epidemiology
Inflammation Mediators
Chronic Infection
Chronic Kidney Disease
title New trends in AA amyloidosis with renal involvement: a single-center experience
title_full New trends in AA amyloidosis with renal involvement: a single-center experience
title_fullStr New trends in AA amyloidosis with renal involvement: a single-center experience
title_full_unstemmed New trends in AA amyloidosis with renal involvement: a single-center experience
title_short New trends in AA amyloidosis with renal involvement: a single-center experience
title_sort new trends in aa amyloidosis with renal involvement a single center experience
topic Amyloid Protein AA
Amyloidosis
Epidemiology
Inflammation Mediators
Chronic Infection
Chronic Kidney Disease
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002025000200302&lng=en&tlng=en
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