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Abstract: FR-PO638

A Case of Tubulointerstitial Nephritis Complicated by Primary Biliary Cirrhosis with Renal Tubular Acidosis and Nephrogenic Diabetes Insipidus

Session Information

Category: Fluid and Electrolytes

  • 902 Fluid and Electrolytes: Clinical


  • Shimizu, Mao, Kita-Harima Medical Center, Kobe, Japan
  • Kitamura, Ken, Kita-Harima Medical Center, Kobe, Japan

Tubulointerstitial nephritis is often caused by drugs, infections, autoimmune disorders. We report a case of tubulointerstitial nephritis associated with primary biliary cirrhosis, which developed renal tubular acidosis and nephrogenic diabetes insipidus.

Case Description

A 50-year-old woman presented with a four-month history of left shoulder pain, back pain, dry mouth, polydipsia, polyuria. She hospitalized for weakness of limbs. Her lab studies revealed serum creatinine 2.72 mg/dL, serum potassium 1.5 mg/dl, and normal anion gap metabolic acidosis which suggested distal and proximal renal tubular acidosis. She had multiple fractures which suggested the presence of osteomalacia. Her urine output exceeded 10L/day, and urine osmolarity was low. After corrected serum potassium weakness of limbs improved, but polyuria did not improve. She was thought to nephrogenic diabetes insipidus because of resistant to antidiuretic hormone. Kidney biopsy was performed to clarify the cause of these findings. The major histologic changes were interstitial infiltration by mainly lymphocytes, tubulitis, and interstitial edema. The presence of antimitochondrial M2 antibodies suggested primary biliary cirrhosis. She was treated with oral steroids and a thiazide diuretic. Her polyuria resolved, and serum creatinine level improved to 0.9 mg/dl.


In conclusion, our patient with tubulointerstitial nephritis and primary biliary cirrhosis had a favorable outcome from oral steroids and a thiazide diuretic. Previous studies have demonstrated Fanconi syndrome in patients with primary biliary cirrhosis, and our patient suggested that tubulointerstitial nephritis complicated by primary biliary cirrhosis could develop renal tubular acidosis and nephrogenic diabetes insipidus.