Carretero Marcelina, Minoletti Sofia Antonella, Aguirre María Adela, Nucifora Elsa Mercedes, Sáez María Sáez, Martínez María Lourdes Posadas
Hospital Italiano de Buenos Aires, Juan Domingo Perón 4190, C1199, Ciudad Autónoma de Buenos Aires, Argentina.
Área de Investigación en Medicina Interna, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina.
J Nephrol. 2024 Nov;37(8):2351-2354. doi: 10.1007/s40620-024-02046-y. Epub 2024 Sep 3.
Two-thirds of patients with immunoglobulin light chain (AL) amyloidosis have renal involvement. The biochemical profile of kidney damage is poorly described.
A cross-sectional study was conducted involving patients diagnosed with AL amyloidosis and renal involvement between January 1, 2010, and April 30, 2022 at the Hospital Italiano de Buenos Aires. Participants were retrospectively identified from the Institutional Amyloidosis Registry. Patients diagnosed with AL amyloidosis and evidence of renal involvement were included. Individuals with other types of amyloidosis were excluded. The selection process involved a thorough review of medical records and registry data to ensure accurate identification and inclusion of eligible participants.
Seventy-seven patients were included. At diagnosis, 90% of the subjects had proteinuria, with a median of 4.3 g/24 h, 61% had renal failure, and 47% presented nephrotic syndrome. Semi-automated urinary electrophoresis revealed 55% with non-selective and 21% with moderately selective glomerular proteinuria. Urine immunofixation indicated 64% with lambda monoclonal free light chains and 12% with kappa. Serum immunofixation demonstrated 48% with lambda monoclonal type and 25% with lambda IgG. At the time of diagnosis of AL amyloidosis, the median age was 66 years (IQR 53-72) and 49% were men. In addition to kidney involvement, other organs were also affected: heart in 53%, gastrointestinal system in 19%, peripheral nervous system in 16%, and liver in 16% of patients.
Our study provides a biochemical profile in renal amyloidosis due to immunoglobulin light chains in a Latin American population. Proteinuria emerged as the most common finding in this cohort with frequent multiorgan involvement.
三分之二的免疫球蛋白轻链(AL)淀粉样变性患者有肾脏受累情况。关于肾脏损害的生化特征描述较少。
在布宜诺斯艾利斯意大利医院进行了一项横断面研究,纳入2010年1月1日至2022年4月30日期间诊断为AL淀粉样变性且有肾脏受累的患者。参与者是从机构淀粉样变性登记处回顾性识别出来的。纳入诊断为AL淀粉样变性且有肾脏受累证据的患者。排除其他类型淀粉样变性的个体。选择过程包括对病历和登记数据进行全面审查,以确保准确识别和纳入符合条件的参与者。
共纳入77例患者。诊断时,90%的受试者有蛋白尿,中位数为4.3 g/24小时,61%有肾衰竭,47%表现为肾病综合征。半自动尿电泳显示55%为非选择性、21%为中度选择性肾小球蛋白尿。尿免疫固定显示64%为λ单克隆游离轻链,12%为κ。血清免疫固定显示48%为λ单克隆型,25%为λ IgG。在诊断AL淀粉样变性时,中位年龄为66岁(四分位间距53 - 72),49%为男性。除肾脏受累外,其他器官也受到影响:53%的患者心脏受累,19%的患者胃肠道系统受累,16%的患者周围神经系统受累,16%的患者肝脏受累。
我们的研究提供了拉丁美洲人群中免疫球蛋白轻链所致肾脏淀粉样变性的生化特征。蛋白尿是该队列中最常见的发现,且常伴有多器官受累。