Tsai Shang-Feng, Wen Mei-Chin, Cheng Chi-Hung, Wu Ming-Ju, Chen Cheng-Hsu, Yu Tung-Min, Chuang Ya-Wen, Huang Shih-Ting, Shu Kuo-Hsiung
Division of Nephrology, Department of Medicine, Taichung Veterans General Hospital, Taiwan.
Intern Med. 2011;50(21):2511-7. doi: 10.2169/internalmedicine.50.5822. Epub 2011 Nov 1.
Although rare, renal amyloidosis is an important diagnosis that bares a significant morbidity and mortality. The purpose of this study was to present current single center experience in the past 28 years.
A total of 6151 kidney biopsies were performed in our hospital from Jan. 1983 to Dec. 2010; 5844 were native kidney biopsies. Cases with a diagnosis of renal amyloidosis were identified through a search of our data bank and the clinical data were retrieved from medical charts. The patients were further classified as AL type and non-AL type. The trend of incidence was analyzed and the clinical features were compared. Risk factors that predict patient mortality were discovered by logistic regression analysis.
Forty out of 5844 native kidney biopsies (0.68%) were identified as renal amyloidosis. Eighteen (45%) were of AL type. The mean age at diagnosis was 62.6 ± 13.4 years. Male was the predominant gender that was involved. Clinically, most (80%) have nephrotic range proteinuria (mean 24 hours urine protein: 6.9 ± 4.7 g). With a mean follow-up duration of 1155 day, the 1-year and 5-year patient survival were 42.9% and 17.9%, respectively. eGFR at biopsy was the sole predictive factor for patient survival.
Senile patients with nephrotic syndrome should elicit the suspicion of renal amyloidosis. The incidence appeared to be increasing and the prognosis poor.
肾淀粉样变性虽罕见，但却是一种具有较高发病率和死亡率的重要诊断。本研究旨在介绍过去28年单中心的经验。
1983年1月至2010年12月，我院共进行了6151例肾活检；其中5844例为原发性肾活检。通过检索数据库确定肾淀粉样变性诊断病例，并从病历中检索临床数据。患者进一步分为AL型和非AL型。分析发病率趋势并比较临床特征。通过逻辑回归分析发现预测患者死亡率的危险因素。
5844例原发性肾活检中有40例（0.68%）被诊断为肾淀粉样变性。18例（45%）为AL型。诊断时的平均年龄为62.6±13.4岁。男性为主要受累性别。临床上，大多数（80%）有肾病范围蛋白尿（平均24小时尿蛋白：6.9±4.7g）。平均随访时间为1155天，1年和5年患者生存率分别为42.9%和17.9%。活检时的估算肾小球滤过率（eGFR）是患者生存的唯一预测因素。
老年肾病综合征患者应怀疑肾淀粉样变性。发病率似乎在上升，预后较差。
