Marques Nídia, Oliveira-Silva Catarina, Pinho Ana, Nunes Ana Teresa, Sampaio Susana, Oliveira Ana, Tavares Isabel
Unidade Local de Saúde São João, Departamento de Nefrologia, Porto, Portugal.
Unidade Local de Saúde de Braga, Departamento de Nefrologia, Braga, Portugal.
J Bras Nefrol. 2025 Apr-Jun;47(2):e20240127. doi: 10.1590/2175-8239-JBN-2024-0127en.
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.
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.
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).
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.
在葡萄牙西北部，AA型淀粉样变性被报道为最常见的淀粉样肾病，但尚不清楚该疾病的发病率和预后是否发生了变化。作者研究了过去40年中肾AA型淀粉样变性患者的流行病学、病因及预后的变化情况。
这是一项回顾性单中心队列研究，纳入了1978年至2019年在葡萄牙西北部经肾活检确诊为AA型淀粉样变性的患者。根据诊断年份将患者分为14年的队列（CA组1978 - 1991年；CB组1992 - 2005年；CC组2006 - 2019年），并分析其临床病程和预后。
共纳入69例AA型淀粉样变性患者。CA组（64%）和CB组（62.7%）的疾病发病率保持稳定，而最近队列（44%，p = 0.027）则显著下降。就诊时的平均年龄从CA组到CC组增加了10岁。总体而言，感染是主要死因，且随时间显著增加（CA组为9.1%，CC组为76.9%，p = 0.002）。三个队列之间的总体生存率和肾脏生存率无显著差异。然而，CC组患者的死亡年龄（61.4岁）高于CA组（52.3岁）。
在过去40年中，AA型淀粉样变性的发病率一直在下降。相比之下，淀粉样肾病的就诊年龄一直在增加。总体和肾脏预后并未改善，但平均预期寿命有所增加，这表明在肾脏及潜在病理的综合管理和支持治疗方面取得了进展。
