Abstract

A 39 year old women with a primary Sjögren syndrome (pSS) had bilateral and multiple nephrolithiasis and nephrocalcinosis due to distal renal tubular acidosis (dRTA), hypercalciuria and hypocitraturia. She had in serum positive antinuclear antibodies with mottled pattern 1/320, totals ENA, Anti-SSA/Ro 52, Anti-SSA/Ro 60 and Anti-SSB-La antibodies. Stones were removed with extracorporeal shock wave lithotripsy satisfactory and were composed of calcium phosphate and calcium oxalate. Metabolic abnormalities were resolved with potassium citrate and hydrochlorothiazide. At two years of follow-up, the patient hadn’t stone recurrence and had normal 24-hour urinary levels of citrate and calcium.

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