Weiner Stefan Markus
Dtsch Med Wochenschr. 2018 Jan;143(2):89-100. doi: 10.1055/s-0043-106563. Epub 2018 Jan 22.
Renal involvement is common and heterogenous in connective tissue diseases and has a main influence on prognosis and mortality. In systemic lupus erythematosus proliferative glomerulonephritis is the most common manifestation, while in primary Sjogren's syndrome interstitial nephritis with tubular dysfunction is the predominant pathological feature. In systemic sclerosis the most serious renal manifestation is scleroderma renal crisis characterized by abrupt onset of hypertension and acute kidney injury associated with an increase in plasma renin activity. Risk factors for scleroderma renal crisis are diffuse cutaneous involvement, treatment with corticosteroids > 15 mg prednisolone/day and treatment with calcineurin inhibitors.Regular measurement of urine sediment, proteinuria-to-urine creatinine ratio, tubular proteinuria, measurement of plasma creatinine, and office as well as home blood pressure monitoring are strongly recommended. Diagnostic kidney biopsy is essential in differentiating the different types of lupus nephritis and renal involvement in sjogren's syndrome.The optimal treatment of lupus nephritis varies with the classification of the morphological findings present on renal biopsy. The treatment of interstitial nephritis in sjogren's syndrome consists of immunosuppression e. g. corticosteroids. Renal tubular acidosis should be corrected by sodium alkali and potassium alkali. Angiotensin-converting enzyme inhibitors play a major role in the treatment of scleroderma renal crisis, they should be continued also in patients progressing to end-stage renal disease.
肾脏受累在结缔组织病中很常见且具有异质性,对预后和死亡率有重大影响。在系统性红斑狼疮中,增殖性肾小球肾炎是最常见的表现,而在原发性干燥综合征中,伴有肾小管功能障碍的间质性肾炎是主要的病理特征。在系统性硬化症中,最严重的肾脏表现是硬皮病肾危象,其特征为高血压突然发作和急性肾损伤,并伴有血浆肾素活性升高。硬皮病肾危象的危险因素包括弥漫性皮肤受累、使用泼尼松龙>15mg/天的皮质类固醇治疗以及使用钙调神经磷酸酶抑制剂治疗。强烈建议定期检测尿沉渣、蛋白尿与尿肌酐比值、肾小管蛋白尿、测定血浆肌酐以及进行门诊和家庭血压监测。诊断性肾活检对于区分不同类型的狼疮性肾炎和干燥综合征中的肾脏受累至关重要。狼疮性肾炎的最佳治疗方法因肾活检时存在的形态学发现分类而异。干燥综合征中间质性肾炎的治疗包括免疫抑制,例如使用皮质类固醇。肾小管酸中毒应通过钠盐和钾盐进行纠正。血管紧张素转换酶抑制剂在硬皮病肾危象的治疗中起主要作用,对于进展至终末期肾病的患者也应继续使用。