da Fonseca Elissa Oliveira, Filho Porphirio Jose Soares, da Silva Licinio Esmeraldo, Caldas Maria Lucia Ribeiro
Hospital Universitario Antonio Pedro, Universidade Federal Fluminense, Rio de Janeiro, Brazil.
J Nephropathol. 2015 Jan;4(1):7-12. doi: 10.12860/jnp.2015.02. Epub 2015 Jan 1.
Renal amyloidosis is one of the main differential diagnoses in the investigation of nephrotic proteinuria in adults, especially elderly patients.
The aim of this article is to contribute to international research with epidemiologic data of renal amyloidosis, given the lack of uniformity described in the literature.
A retrospective study of 37 cases of renal amyloidosis diagnosed by kidney biopsy, between 2000 and 2011, considering epidemiological, clinical and laboratory data.
Subjects aged between 32 and 80 years. Of the 37 cases, 21 (56.8%) were diagnosed as non-light chain (non-AL) renal amyloidosis and 16 (43.2%) as light chain amyloidosis (AL). There was seen an increase in number of both AL and non-AL cases, with a slight predominance in non-AL. The mean 24-hour proteinuria was 5839.0 mg/day. Hematuria was present in 75% of patients. Hypertension was reported in 34% of patients. Acute renal failure, occurred in about 10% of patients, and chronic loss of renal function was present in about 5% at diagnosis.
Renal amyloidosis is a disease of increasing incidence. The forms of clinical presentation proved to be variable, but the presence of proteinuria or nephrotic syndrome in elderly patients should always prompt the suspicion of renal amyloidosis and is a formal indication of renal biopsy.
肾淀粉样变性是成人肾病性蛋白尿，尤其是老年患者肾病性蛋白尿调查中的主要鉴别诊断之一。
鉴于文献中描述缺乏一致性，本文旨在通过肾淀粉样变性的流行病学数据为国际研究做出贡献。
对2000年至2011年间经肾活检确诊的37例肾淀粉样变性病例进行回顾性研究，考虑流行病学、临床和实验室数据。
患者年龄在32岁至80岁之间。37例病例中，21例（56.8%）被诊断为非轻链（非AL）肾淀粉样变性，16例（43.2%）为轻链淀粉样变性（AL）。AL和非AL病例数量均有所增加，非AL略占优势。24小时蛋白尿平均为5839.0毫克/天。75%的患者出现血尿。34%的患者报告有高血压。约10%的患者发生急性肾衰竭，诊断时约5%的患者存在慢性肾功能丧失。
肾淀粉样变性是一种发病率不断上升的疾病。临床表现形式多样，但老年患者出现蛋白尿或肾病综合征时应始终怀疑肾淀粉样变性，这是肾活检的正式指征。
