Sjögren''s Syndrome Associated with Renal Tubular Acidosis, Medullary Sponge Kidney and Multiple Immunological Abnormalities

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  • Sjogren′s syndrome associated with renal tubular acidosis,medullary sponge kidney and multiple immunological abnormalities
  • Sjogren s syndrome associated with rena

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A 20-year-old Japanese female was admitted to hospital with quadriplegia and arthralgia. Three years previous to admission proteinuria and polyarthralgia were evidenced and the following- year erythematous eruptions on both cheeks and upper extremeties, quadriplegia, high fever and stomatitis developed. A hypopotassemia was also present. Since that time she had recurrent attacks of painful swelling of both parotid glands, myalgia, alopecia and nodular erythema. She had also passed urinary stones without concomitant pain or fever. A metabolic acidosis with hypopotassemia was demonstrated in the arterial blood. The urine was alkaline and urine concentrating ability was impaired. Inadequate urinary acidification was evident after administration of ammonium chloride. Such findings supported the diagnosis of renal tubular acidosis (RTA) of the distal type. An intravenous pyelogram (IVP) suggested medullary sponge kidney (MSK). Hypergammaglobulinemia was accompanied by multiple immunological abnormalities such as positive anti-thyroid antibodies and the evidence for anti-nuclear and anti-DNA antibodies. Biopsy specimens of the kidney, salivary and thyroid glands showed small, round cell infiltration in the interstitial tissues. Although sicca syndrome was not prominent, salivary secretion was decreased and a sialectasis was demonstrated by a sialogram. A diagnosis of Sjögren's syndrome was made and following treatment with potassium salt the patient recovered and was discharged. A 3 year follow-up revealed that the patient was well and living a normal life. Discussion was made regarding the etiological relationship between SjÖgren's syndrome, MSK and RTA.

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