干燥综合征的肾脏损害

Renal impairment in Sjogren's syndrome (SS)

结合肾活检及近期检测进展,了解干燥综合征(SS)的肾脏损害。方法 对我科1988～1995年间收住的31例SS肾脏损害患者,进行常规、免疫学、肾小管功能及肾活检等检查。结果 25/31为远端肾小管酸中毒(dRTA),3/25合并肾性尿崩症,2/25合并低钾性麻痹。肾病综合征及肾小球肾炎(GN)分别为5及3例,其中4例肾功能轻度受损。74％患者为高γ球蛋白血症,65％和45％患者抗ss-A、抗ss-B(+)。光镜13例肾标本中9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,2/13为狼疮性肾(LN)Ⅲ及Ⅳ型,2/13为系膜增生型GN。免疫荧光大多阴性,部分标本有免疫球蛋白和补体小球沉积,1例IgG肾间质沉积。19例应用强的松治疗,3例合用CTX冲击,14例缓解,肾功能受损者Cr水平基本降至正常。结论 SS肾脏损害常见,以dRTA和GN主要表现。强的松治疗可减少间质淋巴浆细胞浸润,改善肾功能,纠正RTA。

To further study the renal damage of SS with biopsy and recent determination. Methods 31 patients of SS with renal impairment from 1988 to 1995 were analyzed by routine, immunoassay,tubular function and biopsy examination. Results 25/31 cases presented dRTA, 3/25 with diabetes insipitus and 2/25 with hypokalemic paralysis. Glomerulopathy was occured in 8 of 31 (NS 5, GN 3) and mild renal failure( RF) in 4. Hypergamma-globulinemia was observed in 23 of 31 (74%). About 65% and 45% patients revealed anti ss-A and anti ss-B positive respectively. In 13 renal biopsies, 9 specimens showed CIN with large number of lymphoplasma cells infiltration and tubular atrophy and other 2 specimens revealed LN (type Ⅲ and Ⅳ ) and 2 MPGN. By IF, no positive findings were seen in 7 cases and deposition of IgA, IgM or 63 in some other patients. IgG deposits in the interstitial lymphoplasma cells in 1 patient. 19 patients were treated with prednisone and 3 combined with CTX. 14 cases remitted during the follow-up. In 4 patients with RF, Scr level returned to normal after treatment. Conclusion The renal impairment of SS may be the presenting or predominant feature. Their major clinical manifestations are dRTA and GN. The treatment with prednisone may decrease the infiltration of lymphoplasma cells in the interstitium, improve the renal function and correct RTA.