Renal tubular acidosis type i with prominent hypokalemia and nephrolithiasis as a presentation of sjögren's/systemic lupus erythematosus disease

Anna B. Basok, Yosef S. Haviv, Boris Rogachev, Marina Vorobiov

Research output: Contribution to journalReview articlepeer-review

Abstract

Female patient, suffering from nephrolithiasis, at the age of 32 was admitted for renal colic caused by a stone obstructing UP junction with left hydronephrosis. Nephrostomy was placed, resulting in brisk diuresis. Severe metabolic acidosis with normal anion gap and urine pH of 6.5 was noted. Potassium level dropped to extremely low level (1.6 mEq/L), causing muscle paralysis and respiratory failure, necessitating mechanical ventilation. The patient was treated by potassium chloride infusion, followed by correction of severe metabolic acidosis by sodium bicarbonate. Diagnosis of distal type renal tubular acidosis type I (dRTA) was made based on normal anion gap metabolic acidosis, alkaline urine, hypokalemia, and nephrolithiasis. Five years later, the patient presented with severe hypoxia, lung opacities, and bronchiolitis obliterans organizing pneumonia which was confirmed by bronchoscopy with lung tissue biopsy. Concurrently, the patient presented with dry mouth, pruritus, skin rash with hypocomplementemia, elevated anti-DNA, anti-Ro, and anti-SmAb. Diagnosis of overlap Sjögren's/systemic lupus erythematosus disease was done and treatment by hydroxychloroquine, prednisone, and azathioprine was started. Possible presence of Sjögren's syndrome should be considered in adult patients with unexplained dRTA.

Original languageEnglish
Pages (from-to)247-253
Number of pages7
JournalCase Reports in Nephrology and Dialysis
Volume11
Issue number2
DOIs
StatePublished - 12 Aug 2021

Keywords

  • Bronchiolitis obliterans
  • Renal tubular acidosis type I
  • Sjögren's syndrome

ASJC Scopus subject areas

  • Nephrology

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