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一名患有原发性干燥综合征的孕妇因系膜增生性肾小球肾炎导致急性肾衰竭。

Acute renal failure due to mesangial proliferative glomerulonephritis in a pregnant woman with primary Sjögren's syndrome.

作者信息

Adam Fatma Ulku, Torun Dilek, Bolat Filiz, Zumrutdal Aysegul, Sezer Siren, Ozdemir Fatma Nurhan

机构信息

Department of Nephrology, Baskent University Faculty of Medicine, Adana Teaching and Medical Research Center, Dadaloglu Mah 39/6 PK, 01250 Adana, Yuregir, Turkey.

出版信息

Clin Rheumatol. 2006 Feb;25(1):75-9. doi: 10.1007/s10067-005-1131-8. Epub 2005 May 26.

Abstract

The most common form of renal involvement in Sjögren's syndrome (SS) is tubulointerstitial nephritis. Renal dysfunction is usually mild and subclinical. Glomerulonephritis (GMN) is rare in patients with SS. We report a 28-year-old multigravida patient with primary Sjögren's syndrome (pSS) and associated manifestations, who presented with acute renal failure in the 20th week of her fifth pregnancy. The complaints and clinical findings, positive Schirmer's test, findings of dry eye on ophthalmologic examination, and the salivary gland biopsy were compatible with SS. The patient exhibited no other clinical or laboratory findings indicative of other collagenous disease and/or rheumatoid arthritis. She refused renal biopsy, hesitating for fear of fetal loss; thus, based on the clinical and laboratory findings indicating rapidly progressive GMN and vasculitis, prednisolone, plasmapheresis, and one dose of cyclophosphamide were administered during the pregnancy. Hemodialysis five times weekly was performed. At the 28th week of gestation, she underwent a cesarean section due to early rupture of membranes and fetal distress. A healthy male boy was delivered. The renal biopsy performed 2 weeks after labor revealed mesangial proliferative glomerulonephritis. After the fourth cyclophosphamide treatment, her urinary output increased and she was discharged from the hemodialysis program. She remains in follow-up at our outpatient clinic free of hemodialysis for 4 months. This is the first report of mesangial proliferative GMN requiring dialysis in a pregnant pSS patient that has featured good maternal and fetal outcomes.

摘要

干燥综合征(SS)中最常见的肾脏受累形式是肾小管间质性肾炎。肾功能障碍通常较轻且为亚临床状态。肾小球肾炎(GMN)在SS患者中较为罕见。我们报告一名28岁的多产妇,患有原发性干燥综合征(pSS)及相关表现,在其第五次妊娠的第20周出现急性肾衰竭。患者的症状和临床发现、阳性的Schirmer试验、眼科检查发现的干眼以及唾液腺活检结果均与SS相符。该患者没有其他提示其他胶原性疾病和/或类风湿关节炎的临床或实验室检查结果。她拒绝进行肾活检,因担心胎儿丢失而犹豫不决;因此,基于提示快速进展性GMN和血管炎的临床和实验室检查结果,在孕期给予了泼尼松龙、血浆置换及一剂环磷酰胺治疗。每周进行5次血液透析。妊娠第28周时,因胎膜早破和胎儿窘迫,她接受了剖宫产。产下一名健康男婴。产后2周进行的肾活检显示为系膜增生性肾小球肾炎。在第四次环磷酰胺治疗后,她的尿量增加,停止了血液透析治疗。她在我们的门诊随访4个月,未进行血液透析。这是首例关于妊娠pSS患者出现需要透析的系膜增生性GMN且母婴结局良好的报道。

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