PDF(Pubmed)
Abstract：
Renal tubular acidosis is the principal clinical feature associated with tubulointerstitial nephritis in patients with primary Sjögren\'s syndrome. Renal tubular dysfunction due to interstitial nephritis has been considered the underlying pathophysiology connecting renal tubular acidosis and primary Sjögren\'s syndrome. However, the detailed mechanisms underlying the pathophysiology of renal tubular acidosis in primary Sjögren\'s syndrome is not fully understood.
A 30-year-old woman was admitted with complaints of weakness in the extremities. The patient was hospitalized thirteen years earlier for similar issues and was diagnosed with hypokalemic paralysis due to distal renal tubular acidosis with primary Sjögren\'s syndrome. This diagnosis was based on a positive Schirmer\'s test. Besides, anti-Sjögren\'s syndrome-related antigen A was also detected. Laboratory tests indicated distal RTA; however, a renal biopsy showed no obvious interstitial nephritis. Laboratory tests conducted during the second admission indicated distal renal tubular acidosis. Therefore, a renal biopsy was performed again, which revealed interstitial nephritis. Histological analysis of acid-base transporters revealed the absence of vacuolar type H+-ATPases in the collecting duct. The vacuolar type H+-ATPase was also absent in the past renal biopsy, suggesting that the alteration in acid-base transporters is independent of interstitial nephritis.
This case study demonstrates that vacuolar-type H+-ATPases are associated with distal renal tubular acidosis, and distal renal tubular acidosis precedes interstitial nephritis in patients with primary Sjögren\'s syndrome.
A 30-year-old woman was admitted with complaints of weakness in the extremities. The patient was hospitalized thirteen years earlier for similar issues and was diagnosed with hypokalemic paralysis due to distal renal tubular acidosis with primary Sjögren\'s syndrome. This diagnosis was based on a positive Schirmer\'s test. Besides, anti-Sjögren\'s syndrome-related antigen A was also detected. Laboratory tests indicated distal RTA; however, a renal biopsy showed no obvious interstitial nephritis. Laboratory tests conducted during the second admission indicated distal renal tubular acidosis. Therefore, a renal biopsy was performed again, which revealed interstitial nephritis. Histological analysis of acid-base transporters revealed the absence of vacuolar type H+-ATPases in the collecting duct. The vacuolar type H+-ATPase was also absent in the past renal biopsy, suggesting that the alteration in acid-base transporters is independent of interstitial nephritis.
This case study demonstrates that vacuolar-type H+-ATPases are associated with distal renal tubular acidosis, and distal renal tubular acidosis precedes interstitial nephritis in patients with primary Sjögren\'s syndrome.
摘要：
背景：肾小管酸中毒是原发性干燥综合征患者肾小管间质性肾炎的主要临床特征。间质性肾炎引起的肾小管功能障碍被认为是连接肾小管酸中毒和原发性干燥综合征的潜在病理生理学。然而,原发性干燥综合征肾小管酸中毒病理生理学的详细机制尚不完全清楚.
方法：一名30岁女性因四肢无力而入院。该患者13年前因类似问题住院，并因远端肾小管酸中毒伴原发性干燥综合征而被诊断为低钾性瘫痪。该诊断是基于Schirmer阳性测试。此外,还检测到抗舍格伦综合征相关抗原A。实验室测试表明远端RTA；然而，肾活检未见明显的间质性肾炎。第二次入院期间进行的实验室检查显示远端肾小管酸中毒。因此,再次进行了肾活检，显示间质性肾炎.酸碱转运蛋白的组织学分析显示，收集管中不存在液泡型H-ATPases。在过去的肾活检中也没有空泡型H-ATP酶，提示酸碱转运蛋白的改变与间质性肾炎无关。
结论：本病例研究表明，空泡型H+-ATP酶与远端肾小管酸中毒有关，在原发性干燥综合征患者中，远端肾小管酸中毒先于间质性肾炎。
方法：一名30岁女性因四肢无力而入院。该患者13年前因类似问题住院，并因远端肾小管酸中毒伴原发性干燥综合征而被诊断为低钾性瘫痪。该诊断是基于Schirmer阳性测试。此外,还检测到抗舍格伦综合征相关抗原A。实验室测试表明远端RTA；然而，肾活检未见明显的间质性肾炎。第二次入院期间进行的实验室检查显示远端肾小管酸中毒。因此,再次进行了肾活检，显示间质性肾炎.酸碱转运蛋白的组织学分析显示，收集管中不存在液泡型H-ATPases。在过去的肾活检中也没有空泡型H-ATP酶，提示酸碱转运蛋白的改变与间质性肾炎无关。
结论：本病例研究表明，空泡型H+-ATP酶与远端肾小管酸中毒有关，在原发性干燥综合征患者中，远端肾小管酸中毒先于间质性肾炎。
