Division of Nephrology, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa.
Department of Anatomical Pathology, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa.
BMC Nephrol. 2019 Nov 9;20(1):406. doi: 10.1186/s12882-019-1601-x.
Kidney disease is a serious manifestation of systemic amyloidosis and a major cause of morbidity and mortality. Tuberculosis (TB) occurs up to 27 times more commonly in human immunodeficiency virus (HIV) infected patients and is also an important cause of renal amyloid; there are however no reports of renal amyloidosis in South Africa in the HIV era.
This was a retrospective record review of cases of amyloidosis diagnosed on renal biopsies at our tertiary referral hospital between January 1985 and December 2016.
Forty-six cases of amyloidosis were identified over the study period. The calculated biopsy prevalence was 1.38 per 100 non-transplant renal biopsies (95% Confidence Interval 1.02-1.86). AL amyloidosis was identified in 26 (57%) cases and AA in 20 (43%). The median age at presentation was 51 years and 52% of cases were female. Patients with AA amyloidosis were significantly younger compared to their AL counterparts (age 42 years vs. 58 years, p = < 0.001) and were all significantly non-white. The main clinical presentation was nephrotic syndrome (85%) and 52% of cases also had a serum creatinine value of greater than 120 μmol/L. Of the 20 cases of AA amyloidosis, 12 (60%) were associated with tuberculosis. HIV infection was noted in only two (10%) of the 20 AA cases. Median survival after diagnosis was 2 months.
Amyloidosis is a rare cause of kidney disease and typically presents with nephrotic syndrome. A similar number of AA and AL types were observed, and outcomes are worse in cases of AA amyloid. While TB remains the major underlying disease in this type, HIV infection was infrequent in cases of AA renal amyloidosis.
肾脏疾病是全身性淀粉样变性的严重表现，也是发病率和死亡率的主要原因。结核病（TB）在人类免疫缺陷病毒（HIV）感染者中发生的频率高达 27 倍，也是肾脏淀粉样变性的重要原因；然而，在 HIV 时代，南非尚未有关于肾脏淀粉样变性的报道。
这是对我们的三级转诊医院在 1985 年 1 月至 2016 年 12 月期间通过肾活检诊断的淀粉样变性病例进行的回顾性记录审查。
在研究期间共发现 46 例淀粉样变性病例。计算出的活检患病率为每 100 例非移植性肾活检中有 1.38 例（95%置信区间为 1.02-1.86）。发现 26 例（57%）为 AL 淀粉样变性，20 例（43%）为 AA 淀粉样变性。发病时的中位年龄为 51 岁，52%的病例为女性。与 AL 淀粉样变性患者相比，AA 淀粉样变性患者明显更年轻（年龄 42 岁 vs. 58 岁，p < 0.001），且均为非白人。主要临床表现为肾病综合征（85%），52%的病例血清肌酐值大于 120 μmol/L。20 例 AA 淀粉样变性中有 12 例（60%）与结核病有关。在 20 例 AA 病例中仅发现 2 例（10%）与 HIV 感染有关。诊断后的中位生存时间为 2 个月。
淀粉样变性是肾脏疾病的罕见原因，通常表现为肾病综合征。观察到 AA 和 AL 两种类型的数量相似，AA 淀粉样变性的预后更差。虽然 TB 仍然是此类疾病的主要潜在疾病，但 HIV 感染在 AA 肾淀粉样变性中并不常见。
