一项关于肾淀粉样变性的临床病理发现、电子显微镜检查和分类的 16 年调查。
A 16-year Survey of Clinicopathological Findings, Electron Microscopy, and Classification of Renal Amyloidosis.
机构信息
Department of Pathology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of Epidemiology and Biostatistics, School of Health, Shahrekord University of Medical Sciences, Shahrekord, Iran.
出版信息
Iran J Med Sci. 2021 Jan;46(1):32-42. doi: 10.30476/ijms.2019.82110..
BACKGROUND
Electron microscopy (EM) is a valuable tool in the diagnosis of renal amyloidosis, particularly in the early stages of the disease. In Iran, studies on EM and the clinical features of renal amyloidosis are scarce. The objective of the present study was to survey EM investigations, pathological classifications, and clinical features of renal amyloidosis.
METHODS
This cross-sectional study was performed in Shiraz, Iran, during 2001-2016. Out of 2,770 kidney biopsies, 27 cases with a diagnosis of renal amyloidosis were investigated. EM investigation and six staining procedures for light microscopy (LM) were performed. Two pathological classifications based on glomerular, peritubular, perivascular, and interstitial involvement were made. Finally, the association between these classifications and the clinical features was assessed. Chi-square, Fisher's exact, Independent t test, and Multiple logistic regression analysis were used. P values<0.05 were considered statistically significant.
RESULTS
In 51.9% of the cases, the clinical diagnosis was nephrotic syndrome. Proteinuria and edema were the most prevalent clinical manifestations. The role of EM investigation for diagnosis was graded "necessary" or "supportive" in 48.2% of the patients. In the classification based on glomerular classes, variables such as the mean blood pressure (P=0.003), history of hypertension (P=0.02), creatinine >1.5 (P=0.03), and severe tubular atrophy (P=0.03) were significantly higher in class B (advanced amyloid depositions).
CONCLUSION
EM plays an important role in the diagnosis of renal amyloidosis. EM in conjunction with LM investigation with Congo red staining is recommended, to prevent misdiagnosis of patients with a clinical suspicion of renal amyloidosis. Among different pathological features of renal amyloidosis, the severity of glomerular amyloid depositions had a clear relationship with clinical presentations.
背景
电子显微镜（EM）是诊断肾淀粉样变性的一种有价值的工具，尤其是在疾病的早期阶段。在伊朗，关于 EM 和肾淀粉样变性的临床特征的研究很少。本研究的目的是调查肾淀粉样变性的 EM 研究、病理分类和临床特征。
方法
这是一项在伊朗设拉子进行的横断面研究，时间为 2001 年至 2016 年。在 2770 例肾活检中，有 27 例诊断为肾淀粉样变性。进行了 EM 研究和 6 种用于光镜（LM）的染色程序。根据肾小球、肾小管周围、血管周围和间质受累情况进行了两种病理分类。最后，评估了这些分类与临床特征之间的关联。使用了卡方检验、Fisher 确切检验、独立 t 检验和多因素逻辑回归分析。P 值<0.05 被认为具有统计学意义。
结果
在 51.9%的病例中，临床诊断为肾病综合征。蛋白尿和水肿是最常见的临床表现。EM 研究对诊断的作用在 48.2%的患者中被评为“必要”或“支持”。在基于肾小球分类的分类中，变量如平均血压（P=0.003）、高血压病史（P=0.02）、肌酐>1.5（P=0.03）和严重肾小管萎缩（P=0.03）在 B 类（晚期淀粉样沉积）中显著更高。
结论
EM 在肾淀粉样变性的诊断中起重要作用。建议 EM 与刚果红染色的 LM 研究相结合，以防止对有肾淀粉样变性临床怀疑的患者进行误诊。在肾淀粉样变性的不同病理特征中，肾小球淀粉样沉积的严重程度与临床表现有明确的关系。
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