71例淀粉样变性肾病患者临床及病理特点研究

Clinicopathological features of 71 patients with amyloidosis nephropathy

目的分析本中心淀粉样变性肾病患者的临床及病理特点,旨在为临床诊疗提供更多依据。方法回顾分析1997年12月至2019年12月山东第一医科大学附属省立医院肾内科71例经肾穿刺活检诊断为淀粉样变性患者的临床和病理资料。结果71例患者年龄为(57.2±9.9)岁,50~69岁占71.8%,男女比例为1.7∶1。男性患者年龄、血红蛋白水平高于女性患者,差异具有统计学意义(P<0.05)。血肌酐升高组患者血红蛋白、血清钙显著低于肌酐正常组(P<0.05),而尿素氮、胱抑素C、β2-微球蛋白、尿酸等指标显著高于肌酐正常组患者(P<0.05)。淀粉样物质主要沉积于肾小球系膜区(96.2%)、毛细血管壁(28.3%)和间质小血管壁(47.2%),小部分沉积于内皮下(1.9%)、肾小管(15.1%)或肾间质(5.7%)。血肌酐升高组患者肾小球淀粉样物质大量沉积比率更高(P=0.032),且肾小管萎缩>10%比率更高(P=0.024),而大量蛋白尿与非大量蛋白尿患者组间临床及病理指标差异均无统计学意义。结论淀粉样变性肾病好发于中老年,以老年男性患者多见。淀粉样变性肾病患者以肾小球损害为主,肾小球淀粉样变的程度与确诊时的肾功能有关,而与蛋白尿水平无关。

Objective To explore the clinicopathological features of patients with amyloidosis nephropathy to provide more rationales for clinical diagnosis and treatment.Methods From December 1997 to December 2019,clinicopathological data were retrospectively reviewed for 71 patients with amyloidosis diagnosed by renal biopsy.Results The mean age was(57.2±9.9)years,71.8%were aged 50 to 69 years and male-to-female ratio was 1.7∶1.The age and hemoglobin levels of males were higher than those of females.And the differences were statistically significant(P<0.05).Hemoglobin and serum calcium in patients with higher serum creatinine(Scr)were significantly lower(P<0.05)while urea nitrogen,cystatin C,β2-microglobulin and uric acid were significantly higher than those with normal creatinine(P<0.05).In a declining order,amyloid was deposited in glomerular mesangial area(96.2%),capillary wall(28.3%),interstitial small vessel wall(47.2%),tubules(15.1%),renal interstitium(5.7%)and subendothelium(1.9%).In group of higher Scr,the rates of amyloid deposition were both higher in glomeruli(P=0.032)and tubular atrophy>10%(P=0.024).However,no significant difference existed in clinicopathological parameters between patients with nephrotic-range proteinuria and non-nephrotic-range proteinuria.Conclusion Amyloidosis nephropathy occurs predominantly in middle-aged and elderly males.Glomerular damage is common in patients with amyloidosis nephropathy.And the degree of glomerular amyloidosis in renal tissue is associated with renal insufficiency rather than the level of urinary protein.