Engineer Divyesh P, Kute Vivek B, Patel Himanshu V, Shah Pankaj R
Department of Nephrology and Clinical Transplantation, Institute of Kidney Diseases and Research Centre, Dr. H. L. Trivedi Institute of Transplantation Sciences, Ahmedabad, Gujarat, India.
Saudi J Kidney Dis Transpl. 2018 Sep-Oct;29(5):1065-1072. doi: 10.4103/1319-2442.243966.
The kidney is the most common organ involved in systemic amyloidosis. We aimed to study etiology and clinicopathological profile of renal amyloidosis. This was a retrospective study of 40 consecutive adult patients with biopsy-proven renal amyloidosis evaluated over a period of two years. Emphasis was given to describing the clinical presentation, renal function, proteinuria, type of amyloidosis, and its etiology. Mean age of the study cohort was 44 ± 15 years (with a male-to-female ratio of 3:1). Amyloid A (AA) amyloidosis was the most common type of amyloidosis observed in 72.5% of cases. Amyloid light chain (AL) amyloidosis accounted for 17.5% of cases, and the rest remained undetermined. AA amyloidosis had widespread age distribution while AL amyloidosis was confined to those >40 years. Proteinuria was the most common renal manifestation observed in all patients. Nephrotic syndrome was seen in 70% of patients. Mean 24 h proteinuria was 6.4 g. Renal failure was the second most common manifestation seen in 70% of patients, of whom 21.4% required hemodialysis. Tuberculosis (TB) accounted for 90% cases of AA amyloidosis. The most prevalent form was pulmonary TB while the rest accounted for by rheumatoid arthritis and bronchiectasis. Among patients with TB induced amyloidosis, 61.5% had received adequate treatment for TB in the past. All patients with AL amyloidosis had nephrotic range proteinuria, five had renal failure out of which two required dialysis. Cardiac involvement was seen in two patients. AA amyloidosis was the most common type of renal amyloidosis in the present study and pulmonary TB was the most common etiology.
肾脏是系统性淀粉样变性最常累及的器官。我们旨在研究肾淀粉样变性的病因及临床病理特征。这是一项回顾性研究，对连续40例经活检证实为肾淀粉样变性的成年患者进行了为期两年的评估。重点描述了临床表现、肾功能、蛋白尿、淀粉样变性类型及其病因。研究队列的平均年龄为44±15岁（男女性别比为3:1）。淀粉样蛋白A（AA）淀粉样变性是最常见的淀粉样变性类型，见于72.5%的病例。淀粉样轻链（AL）淀粉样变性占病例的17.5%，其余病例类型未明确。AA淀粉样变性年龄分布广泛，而AL淀粉样变性局限于40岁以上人群。蛋白尿是所有患者中最常见的肾脏表现。70%的患者出现肾病综合征。平均24小时蛋白尿为6.4g。肾衰竭是第二常见的表现，见于70%的患者，其中21.4%需要血液透析。结核病（TB）占AA淀粉样变性病例的90%。最常见的形式是肺结核，其余由类风湿性关节炎和支气管扩张症引起。在结核病诱发淀粉样变性的患者中，61.5%过去曾接受过充分的抗结核治疗。所有AL淀粉样变性患者均有肾病范围的蛋白尿，5例出现肾衰竭，其中2例需要透析。2例患者出现心脏受累。AA淀粉样变性是本研究中最常见的肾淀粉样变性类型，肺结核是最常见的病因。
