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[原发性干燥综合征的肾脏受累:26例临床病理研究]

[Renal involvement in primary Sjögren's syndrome: a clinicopathological study of 26 cases].

作者信息

Yang J, Li X, Huang Q

机构信息

Department of Internal Medicine, Peking Union Medical College Hospital, Beijing.

出版信息

Zhonghua Nei Ke Za Zhi. 1997;36(1):28-31.

PMID:9812558
Abstract

Previous study suggested that renal involvement in primary sjögren's syndrome (SS) was rare and renal damage in SS cases has not been reported in Chinese literature. In this study the clinical and pathological features in 26 primary SS patients (23 female and 3 male, with an average age of 40.9 years) were analyzed. The spectrum of renal disorders in these patients was as follows: renal tubular acidosis (20/26), hypokelemia (18/26), hypocalcemia (14/26), mild proteinuria (less than 1.5 g/d, 12/26), massive proteinuria (more than 1.5 g/d, 9/26, including 5 nephrotic syndrome), and renal insufficiency (5/26). Light microscopy (LM) might show acute or chronic interstitial nephritis (IN, 10/26), or IN accompanied with focal glomerulonephritis (FGN, 4/26), focal segmental glomerulosclerosis (FSGS, 4/26), membranous nephropathy (MN, 3/26), diffuse mesangial proliferative glomerulonephritis (MePGN, 1/26) and membranoproliferative glomerunephritis (MPGN, 1/26) in these SS patients. A variable degree of interstitial lesion was also noted in all patients with glomerular damage. Immunofluorescent examination revealed deposits of IgG (9/24), IgA (8/24), IgM (7/24), C3 (10/24) and C1q (2/24) in granular distribution on the glomerular basement membrane (GBM) or focal masangium and focal deposits of IgG and C3 (7/24) on the tubular basement membrane (TBM). These results suggested that renal tubular acidosis is the most common finding in Chinese SS patients with renal involvement, but glomerular damage is not uncommon. Although IN was the major pathological manifestation in SS patients, FGN, FSGS, MN, MePGN and MPGN might also be found. The exact incidence of secondary glomerular disease in primary SS needs to be studied further.

摘要

既往研究提示,原发性干燥综合征(SS)累及肾脏较为罕见,且中文文献中尚未报道过SS病例的肾损害情况。本研究分析了26例原发性SS患者(23例女性,3例男性,平均年龄40.9岁)的临床和病理特征。这些患者的肾脏疾病谱如下:肾小管酸中毒(20/26)、低钾血症(18/26)、低钙血症(14/26)、轻度蛋白尿(<1.5 g/d,12/26)、大量蛋白尿(>1.5 g/d,9/26,其中5例为肾病综合征)以及肾功能不全(5/26)。光镜检查(LM)可能显示急性或慢性间质性肾炎(IN,10/26),或IN伴局灶性肾小球肾炎(FGN,4/26)、局灶节段性肾小球硬化(FSGS,4/26)、膜性肾病(MN,3/26)、弥漫性系膜增生性肾小球肾炎(MePGN,1/26)和膜增生性肾小球肾炎(MPGN,1/26)。在所有有肾小球损害的患者中也观察到了不同程度的间质病变。免疫荧光检查显示,IgG(9/24)、IgA(8/24)、IgM(7/24)、C3(10/24)和C1q(2/24)呈颗粒状分布于肾小球基底膜(GBM)或局灶系膜区,IgG和C3(7/24)呈局灶性沉积于肾小管基底膜(TBM)。这些结果提示,肾小管酸中毒是中国有肾脏受累的SS患者最常见的表现,但肾小球损害也并不少见。虽然IN是SS患者的主要病理表现,但也可能发现FGN、FSGS、MN、MePGN和MPGN。原发性SS继发肾小球疾病的确切发病率有待进一步研究。

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