84例干燥综合征合并肾脏损害的临床与病理分析

Clinical and pathologic analysis of Sjgren′s syndrome with renal impairment :a report of 84 cases

目的 结合肾活检及相关实验室检测结果 ,进一步探讨干燥综合征 (SS)肾脏损害的临床及病理特征。方法 对我科 1993～ 1999年间收住的 84例SS肾脏损害患者 ,进行常规免疫学、肾小管功能及部分肾活检检查。结果 5 5 / 84例为肾小管性酸中毒 (RTA) ,5 / 5 5例合并肾性尿崩症 ,3/ 5 5例合并低钾性麻痹 ;表现为肾病综合征及肾小球肾炎 (GN)分别为 12及 10例 ;14/ 84例有轻度肾功能衰竭。 6 9.1%的患者为高γ球蛋白血症 ,6 4.3%和 44 .1%的患者抗SS A和抗SS B(+)。肾活体组织检查 37例 ,光镜下 2 1例为慢性间质性肾炎伴间质大量淋巴、浆细胞浸润及小管萎缩 ,10 / 37例为狼疮性肾炎 (LN) ,5例为系膜增生性肾炎。免疫荧光检查大多阴性 ,部分标本肾小球有免疫球蛋白和补体沉积 ,1例IgG肾病患者有间质沉积。 2 5例应用泼尼松及环磷酰胺 (CTX)冲击治疗。 14例肾功能受损者血清肌酐 (Cr)水平基本降至正常。结论 SS肾脏损害常见 ,以RTA和GN为主要表现 ,泼尼松治疗可以减少间质淋巴细胞和浆细胞浸润 ,改善肾功能 。

Objective To further study the renal damage of Sjgren′s syndrome (SS)and its clinical and pathologic characteristics with biopsy and recent technology. Methods 84 patients of SS with renal impairment from 1993 to 1999 were analyzed by routine, immunoassay, tubular function and biopsy examination. Results 55 of the 84 cases presented with renal tubular acidosis (RTA), 5 with diabetes insipidus and 3 with hypokalemic paralysis. Glomerulopathy occurred in 22 cases (nephrotic syndrome 12, glomerulonephritis 10) and mild renal failure (RF)was found in 14. 69.1% of the patients had hypergamma globulinemia. 64.3% and 44.1% of the patients showed positive anti SS A and anti SS B. In 37 renal biopsy specimens 21 showed chronic interstitial nephritis (CIN) with extensive lymphoplasmic cell infiltration and tubular atrophy. 10 of the 37 specimens revealed lupus nephritis (LN, type III and IV) and 5 mesangial proliferative glomerulonephritis (MsPGN). With immunoflurescence tests, no positive findings were seen in most of the specimens, but deposition of IgA, IgM or C 3 was seen in some patients. IgG deposits in the interstitial lymphoplasmic cells were found in 1 patient. 25 patients were treated with prednisone combined with cytoxan (CTX). In 14 patients with renal failure, serum creatinine level returned to normal after treatment. Conclusion Renal impairment may be the presenting or predominant feature in SS. The major clinical manifestations are RTA and GN. Treatment with prednisone may decrease the infiltration of lymphoplasmic cells in the interstitium, improve the renal function and correct RTA.