a Department of Nephrology , Ankara University School of Medicine , Ankara , Turkey.
Amyloid. 2018 Jun;25(2):115-119. doi: 10.1080/13506129.2018.1474733. Epub 2018 May 18.
In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis.
This is a retrospective study involving 53 patients who were diagnosed with AA amyloidosis by kidney biopsy from 2006 to 2016. In all patients, kidney biopsies were performed due to asymptomatic proteinuria, nephrotic syndrome and/or renal insufficiency. The patients were separated into two groups on the basis of age (group I: ≥60 years and group II: <60 years). Outcomes of patients in terms of the requirement of renal replacement therapy and mortality were recorded.
In patients with group I, the causes of AA amyloidosis were as follows: FMF 16 (50%), bronchiectasis 7 (23%), chronic osteomyelitis 2 (6%), inflammatory bowel disease 2 (6%), rheumatoid arthritis 2 (6%), ankylosing spondylitis 1 (3%) and unknown aetiology 2 (6%). The underlying disorders of AA amyloidosis in group II patients were as follows: FMF 17 (81%), Behcet's disease 1 (5%) and unknown aetiology 3 (14%). No statistically significant differences were detected between two groups with regard to systolic and diastolic blood pressures, albumin, proteinuria and lipids. The combination of chronic kidney disease and nephrotic syndrome was the most common clinical presentation in group I (73%) and group II (43%) (p = .05). Compared to the group II, estimated glomerular filtration rate was significantly lower in group I at the time of kidney biopsy (p = .003). At 12-month follow-up, 61% of the group I and 33% of the group II developed end-stage kidney disease requiring dialysis, while 11% of the group I died.
Our results indicated that renal AA amyloidosis is a rare disease in advanced age patients. At baseline and follow-up period, advanced age patients had worse kidney disease and outcomes.
在土耳其进行的淀粉样蛋白 A (AA) 淀粉样变性的流行病学研究中,最常见的病因是家族性地中海热 (FMF),且通常发生在年轻人群中。然而,对于年龄较大的患者中肾 AA 淀粉样变性的病因、临床表现和预后,目前尚无足够的数据。本研究旨在探讨经肾活检证实的年龄在 60 岁及以上的成人肾 AA 淀粉样变性患者的人口统计学、临床表现、病因和结局。
这是一项回顾性研究,纳入了 2006 年至 2016 年间因无症状蛋白尿、肾病综合征和/或肾功能不全行肾活检诊断为 AA 淀粉样变性的 53 例患者。所有患者均因肾活检而被诊断为 AA 淀粉样变性。根据年龄将患者分为两组(I 组:年龄≥60 岁;II 组:年龄<60 岁)。记录患者在接受肾脏替代治疗和死亡方面的结局。
在 I 组患者中,AA 淀粉样变性的病因如下:FMF 16 例(50%)、支气管扩张症 7 例(23%)、慢性骨髓炎 2 例(6%)、炎症性肠病 2 例(6%)、类风湿关节炎 2 例(6%)、强直性脊柱炎 1 例(3%)和病因不明 2 例(6%)。II 组患者的 AA 淀粉样变性的基础疾病如下:FMF 17 例(81%)、白塞病 1 例(5%)和病因不明 3 例(14%)。两组间收缩压和舒张压、白蛋白、蛋白尿和血脂无统计学差异。慢性肾脏病和肾病综合征合并是 I 组(73%)和 II 组(43%)最常见的临床表现(p=0.05)。与 II 组相比,I 组在肾活检时估算的肾小球滤过率明显更低(p=0.003)。在 12 个月的随访中,I 组有 61%和 II 组有 33%的患者进展为需要透析的终末期肾病,而 I 组有 11%的患者死亡。
我们的结果表明,肾 AA 淀粉样变性在老年患者中较为少见。在基线和随访期间,老年患者的肾脏疾病和结局更差。