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肉芽肿性间质性肾炎

Granulomatous interstitial nephritis.

作者信息

Joss Nicola, Morris Scott, Young Barbara, Geddes Colin

机构信息

Renal Unit, Western Infirmary, Glasgow, G11 6NT, Scotland.

出版信息

Clin J Am Soc Nephrol. 2007 Mar;2(2):222-30. doi: 10.2215/CJN.01790506. Epub 2007 Jan 17.

DOI:10.2215/CJN.01790506
PMID:17699417
Abstract

Granulomatous interstitial nephritis (GIN) is a rare histologic diagnosis. This series reports the presenting features, associated conditions, treatment, and outcome of patients with a diagnosis of GIN in Glasgow during a 15-yr period and compares this with the available literature. Eighteen cases were identified: Five cases were associated with sarcoidosis, two were associated with tubulointerstitial nephritis and uveitis, two were associated with medication, and nine were idiopathic. Patients presented with advanced renal failure (median estimated creatinine clearance 21 ml/min) and minimal proteinuria (urine albumin-to-creatinine ratio 9.9 mg/mmol). Sixteen patients were treated with prednisolone for a mean of 25 mo. Six patients relapsed with reduction in prednisolone dosage, and four patients required steroid-sparing agents. During the mean follow-up of 45 mo, renal function improved or stabilized in 17 patients; the rate of improvement in renal function was most marked in the first year after diagnosis with a gain in function of +1.9 ml/min per mo. The median estimated creatinine clearance at final visit was 56 ml/min. One patient required renal replacement therapy at diagnosis but recovered renal function with treatment. No patient required long-term renal replacement therapy. There was no correlation between the degree of fibrosis or inflammation on biopsy and renal outcome, and the features on biopsy did not help to determine the cause of GIN. GIN is a treatable cause of renal failure that highlights the value of renal biopsy in patients who present with renal failure even when there is minimal proteinuria. The rarity of GIN demonstrates the need for systematic data collection.

摘要

肉芽肿性间质性肾炎(GIN)是一种罕见的组织学诊断。本系列报告了格拉斯哥15年间诊断为GIN的患者的临床表现、相关疾病、治疗及预后,并与现有文献进行了比较。共确定了18例患者:5例与结节病相关,2例与肾小管间质性肾炎和葡萄膜炎相关,2例与药物相关,9例为特发性。患者表现为晚期肾衰竭(估计肌酐清除率中位数为21 ml/min)和微量蛋白尿(尿白蛋白与肌酐比值为9.9 mg/mmol)。16例患者接受了泼尼松龙治疗,平均治疗25个月。6例患者在泼尼松龙剂量减少时复发,4例患者需要使用免疫抑制剂。在平均45个月的随访中,17例患者的肾功能得到改善或稳定;肾功能改善率在诊断后的第一年最为显著,每月功能增加1.9 ml/min。末次随访时估计肌酐清除率中位数为56 ml/min。1例患者在诊断时需要肾脏替代治疗,但经治疗后肾功能恢复。没有患者需要长期肾脏替代治疗。活检时的纤维化或炎症程度与肾脏预后之间没有相关性,活检特征也无助于确定GIN的病因。GIN是肾衰竭的一种可治疗病因,突出了肾活检在即使有微量蛋白尿的肾衰竭患者中的价值。GIN的罕见性表明需要系统地收集数据。

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