Kidney Week

Abstract: FR-PO572

Renal Proximal Tubular Dysfunctions in Primary Biliary Cholangitis

Session Information

• Fluid, Electrolyte, and Acid-Base Disorders: Clinical
  November 04, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
  Abstract Time: 10:00 AM - 12:00 PM

Category: Fluid‚ Electrolyte‚ and Acid-Base Disorders

• 1002 Fluid‚ Electrolyte‚ and Acid-Base Disorders: Clinical

Authors

• Shi, Xiaoxiao, Department of Nephrology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China

Xiaoxiao Shi,

• Guo, Tianchen, Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China

Tianchen Guo,

• Wen, Yubing, Department of Nephrology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China

Yubing Wen,

• Li, Xuemei, Department of Nephrology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China

Xuemei Li,

• Chen, Limeng, Department of Nephrology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China

Limeng Chen,

Background

Renal involvement of primary biliary cholangitis (PBC) always presents as distal renal tubular acidosis. Proximal tubular (PT) dysfunctions in PBC have been rarely reported and their clinicopathological characteristics and renal prognosis remain unclear.

Methods

We recruited 935 inpatients of PBC from February 2003 to July 2021 and identified 11 cases with PT dysfunctions (PBC-PT). Their medical document, kidney pathology, and follow-up data were retrospectively reviewed and analyzed.

Results

The 11 PBC-PT patients were mainly middle-aged (57.8±5.2 years) females (81.8%) with a mean estimated glomerular filtration rate (eGFR) level of 46.54±23.03 ml/min/1.73m². They showed different degrees of PT dysfunctions, including hyperuricosuria (80.0%), hypouricemia (63.6%), normoglycemic glycosuria (63.6%), generalized aminoaciduria (62.5%), hyperphosphaturia (60.0%), and hypophosphatemia (54.5%). Compared to the PBC patients with non-proximal tubulointerstitial injuries, the PBC-PT patients had an older age at diagnosis and a lower ratio of severe hypokalemia. Their kidney pathology showed tubulointerstitial nephritis with lymphoplasmacytic infiltrates, brush border defects, and proximal tubulitis. After glucocorticoids (GCs) treatment, we observed the recovery of hypophosphatemia, hypouricemia, renal glycosuria, and the significant improvement of eGFR (43.24±19.60 ml/min/1.73m² vs. 55.02±21.14 ml/min/1.73m², P=0.028).

Conclusion

We reported the largest single-center cases series of PBC-PT. The PT dysfunctions were not rare in PBC patients, and GCs treatment improved the eGFR and tubular functions.

Funding

• Government Support – Non-U.S.