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一名远端肾小管酸中毒患者合并干燥综合征相关间质性肾炎和低钾性肾病

Coexistence of Sjögren's Syndrome-associated Interstitial Nephritis and Hypokalemic Nephropathy in a Patient with Distal Renal Tubular Acidosis.

作者信息

Matsui Rena, Gojo Maika, Odajima Kohei, Asakawa Shinichiro, Arai Shigeyuki, Yamazaki Osamu, Tamura Yoshifuru, Numakura Satoe, Oshima Yasutoshi, Ohashi Ryuji, Shibata Shigeru, Fujigaki Yoshihide

机构信息

Department of Internal Medicine, Teikyo University School of Medicine, Japan.

Department of Pathology, Teikyo University Hospital, Japan.

出版信息

Intern Med. 2025 Apr 1;64(7):1067-1074. doi: 10.2169/internalmedicine.4152-24. Epub 2024 Aug 28.

DOI:10.2169/internalmedicine.4152-24
PMID:39198164
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12021504/
Abstract

A 42-year-old woman presented with muscle weakness and hypokalemic distal renal tubular acidosis (dRTA). Investigations revealed concurrent Sjögren's syndrome (SS) and Hashimoto's thyroiditis contributing to hypokalemic dRTA. A renal biopsy revealed focal tubulointerstitial nephritis (TIN) suggestive of SS-related renal involvement, along with distinctive ischemic glomerular changes and tubular alterations consistent with hypokalemic nephropathy. Rapid improvement in tubular injury markers and hypobicarbonemia followed potassium supplementation, suggesting that hypokalemia contributed to proximal tubular injury. This case underscores the diagnostic challenge posed by the simultaneous presence of TIN and hypokalemic nephropathy, potentially masking hypokalemic nephropathy in patients with hypokalemic dRTA secondary to SS-TIN.

摘要

一名42岁女性出现肌肉无力和低钾性远端肾小管酸中毒（dRTA）。检查发现同时患有干燥综合征（SS）和桥本甲状腺炎，这导致了低钾性dRTA。肾活检显示局灶性肾小管间质性肾炎（TIN），提示与SS相关的肾脏受累，同时伴有独特的缺血性肾小球改变和与低钾性肾病一致的肾小管改变。补充钾后，肾小管损伤标志物和低碳酸氢血症迅速改善，表明低钾血症导致近端肾小管损伤。该病例强调了TIN和低钾性肾病同时存在所带来的诊断挑战，这可能会掩盖继发于SS - TIN的低钾性dRTA患者的低钾性肾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/7417710f7b0a/1349-7235-64-1067-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/6740cc1380a2/1349-7235-64-1067-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/7fca5baadc12/1349-7235-64-1067-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/68ec9c1a4cce/1349-7235-64-1067-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/b32205945134/1349-7235-64-1067-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/7417710f7b0a/1349-7235-64-1067-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/6740cc1380a2/1349-7235-64-1067-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/7fca5baadc12/1349-7235-64-1067-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/68ec9c1a4cce/1349-7235-64-1067-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/b32205945134/1349-7235-64-1067-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bda2/12021504/7417710f7b0a/1349-7235-64-1067-g005.jpg

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本文引用的文献

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Renal calcification in children with renal tubular acidosis: What a paediatrician ‎should ‎know‎.肾小管酸中毒患儿的肾钙化：儿科医生应了解的知识
World J Clin Pediatr. 2023 Dec 9;12(5):295-309. doi: 10.5409/wjcp.v12.i5.295.
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Renal tubular acidosis without interstitial nephritis in Sjögren's syndrome: a case report and review of the literature.干燥综合征合并肾小管性酸中毒而无间质性肾炎：病例报告及文献复习。
BMC Nephrol. 2023 Aug 15;24(1):237. doi: 10.1186/s12882-023-03290-3.
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Recurrent hypokalemic paralysis in hypothyroidism.甲状腺功能减退症中的复发性低钾性麻痹。
Am J Med Sci. 2023 May;365(5):462-469. doi: 10.1016/j.amjms.2023.01.009. Epub 2023 Feb 6.
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Potassium and the kidney: a reciprocal relationship with clinical relevance.钾与肾脏：具有临床相关性的相互关系。
Pediatr Nephrol. 2022 Oct;37(10):2245-2254. doi: 10.1007/s00467-022-05494-5. Epub 2022 Feb 23.
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Renal Disease in Primary Sjögren's Syndrome.原发性干燥综合征中的肾脏疾病
Rheumatol Ther. 2021 Mar;8(1):63-80. doi: 10.1007/s40744-020-00264-x. Epub 2020 Dec 24.
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Kidney Disease Associated With Anorexia Nervosa: A Case Series With Kidney Biopsies.神经性厌食症相关肾病：肾活检病例系列
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Multicellular vacuoles in hypokalemic nephropathy.低钾血症性肾病中的多细胞空泡
Kidney Int. 2020 Mar;97(3):618. doi: 10.1016/j.kint.2019.09.010.
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Hashimotos' thyroiditis: Epidemiology, pathogenesis, clinic and therapy.桥本甲状腺炎：流行病学、发病机制、临床与治疗。
Best Pract Res Clin Endocrinol Metab. 2019 Dec;33(6):101367. doi: 10.1016/j.beem.2019.101367. Epub 2019 Nov 26.
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Hypokalemic Nephropathy.低钾血症性肾病
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Sjögren's syndrome: state of the art on clinical practice guidelines.干燥综合征：临床实践指南的最新进展
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