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狼疮性肾炎中的指纹状及其他有组织沉积物。

Fingerprint and other organised deposits in lupus nephritis.

作者信息

Hvala A, Kobenter T, Ferluga D

机构信息

Institute of Pathology, Faculty of Medicine, University of Ljubljana, Slovenia.

出版信息

Wien Klin Wochenschr. 2000 Aug 25;112(15-16):711-5.

Abstract

A prominent feature of lupus glomerulonephritis is extracellular, predominantly homogeneous electron dense deposits. Fingerprint-like deposits have been reported in 6 to 10% of cases. On electron microscopy, we studied the frequency and characteristics of organised deposits in 227 kidney tissue samples obtained by biopsy in 185 patients with systemic lupus erythematosus (SLE). Fingerprint forms of deposits were demonstrated in 34 biopsies of 32 patients (17.3%). In the control group of 626 kidney biopsies of patients with primary renal and systemic diseases other than SLE, no fingerprint deposits were found. In 227 kidney biopsy samples, fingerprint deposits were found to be associated with mesangial (8.8%), mesangial-subendothelial (3.8%), subepithelial (28.6%), mesangial-subepithelial (11.1%) and mesangial-transmembranous (19.4%) glomerular deposit distribution patterns. They were demonstrated more often at different locations along the peripheral capillary glomerular basal membrane (77.4%) than within the mesangial matrix (43.7%). In extraglomerular locations, fingerprint deposits were present in the interstitium in 8.8%, along the tubular in 23.5% and peritubular capillary basal membrane in 20.5%, in the wall of the arterioles in 64.7% and in the juxtaglomerular apparatus in 18.2% of biopsies. Organised fingerprint deposits consisted of semicircular dark and light lines, each with a diameter of about 10 to 15 nm. Unilaterally, spiky processes at a periodicity of 10 to 15 nm were seen. Among 14 of 185 SLE patients with cryoglobulinemia, fingerprint deposits were demonstrated in only 2 patients. We conclude that fingerprint deposits are characteristic, diagnostically relevant for SLE and represent morphologically a homogeneous group of organised deposits unrelated to cryoglobulins. In 3 SLE patients, 20 to 100 nm tubules and in 2 SLE patients, 10 and 18 nm Congo red negative fibrils were found. By their morphology and their structural characteristics, the tubules and fibrils resemble the tubules in primary immunotactoid glomerulopathy and fibrils in primary fibrillary glomerulonephritis.

摘要

狼疮性肾小球肾炎的一个显著特征是细胞外、主要为均匀的电子致密沉积物。指纹样沉积物在6%至10%的病例中已有报道。在电子显微镜下,我们研究了185例系统性红斑狼疮(SLE)患者经活检获得的227份肾组织样本中有序沉积物的频率和特征。在32例患者的34份活检标本中发现了指纹样沉积物形式(17.3%)。在除SLE以外的原发性肾脏和全身性疾病患者的626份肾活检对照组中,未发现指纹样沉积物。在227份肾活检样本中,发现指纹样沉积物与系膜(8.8%)、系膜-内皮下(3.8%)、上皮下(28.6%)、系膜-上皮下(11.1%)和系膜-跨膜(19.4%)肾小球沉积物分布模式相关。它们更多地出现在沿肾小球外周毛细血管基底膜的不同位置(77.4%),而不是系膜基质内(43.7%)。在肾小球外位置,指纹样沉积物在8.8%的活检标本的间质中、23.5%的肾小管周围、20.5%的肾小管周围毛细血管基底膜、64.7%的小动脉壁和18.2%的肾小球旁器中出现。有序的指纹样沉积物由直径约10至15nm的半圆形明暗线组成。单侧可见周期性为10至15nm的尖刺状突起。在185例SLE合并冷球蛋白血症的患者中,仅2例患者发现了指纹样沉积物。我们得出结论,指纹样沉积物具有特征性,对SLE具有诊断意义,并且在形态学上代表一组与冷球蛋白无关的均匀的有序沉积物。在3例SLE患者中发现了20至100nm的小管,在2例SLE患者中发现了10和18nm的刚果红阴性纤维。根据其形态和结构特征,这些小管和纤维类似于原发性免疫触须样肾小球病中的小管和原发性纤维性肾小球肾炎中的纤维。

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