Journal Article

Clinical and morphological features of kidney involvement in primary Sjögren's syndrome

Nicola Bossini, Silvana Savoldi, Franco Franceschini, Simone Mombelloni, Manuela Baronio, Ilaria Cavazzana, Battista Fabio Viola, Brunella Valzorio, Consuela Mazzucchelli, Roberto Cattaneo, Francesco Scolari and Rosario Maiorca

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 16, issue 12, pages 2328-2336
Published in print December 2001 | ISSN: 0931-0509
Published online December 2001 | e-ISSN: 1460-2385 | DOI:
Clinical and morphological features of kidney involvement in primary Sjögren's syndrome

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Background. Primary Sjögren's syndrome is a connective tissue disorder affecting primarily the lacrimal and salivary glands, resulting in xerophtalmia and xerostomia. Extraglandular manifestations are frequent and may include renal involvement.

Methods. We studied the prevalence and nature of kidney involvement in 60 Italian patients with primary Sjögren's syndrome, diagnosed according to the European classification criteria. The following renal laboratory tests were performed in all patients: electrolytes in serum and in 24‐h urine, creatinine in serum and in 24‐h urine, venous pH and HCO3, urinalysis, urine culture, urinary osmolality and urine pH. A water deprivation test was performed in patients with morning urine osmolalities below the reference values adjusted for age. An oral ammonium chloride loading test was performed in patients with urine pH above 5.5 from morning samples. Renal biopsy was performed in patients with renal involvement.

Results. Sixteen patients (27%) had laboratory evidence of tubular and/or glomerular dysfunction. A variable degree of creatinine clearance reduction was found in eight patients (13%); frank distal tubular acidosis in three (5%); hypokalaemia in four (7%); and pathological proteinuria in 12 (20%). Urine concentrating capacity was defective in 10 out of 48 (21%) tested patients. Only four patients presented with overt clinical manifestations, including hypokalaemic tetraparesis (1), nephrotic syndrome (2), recurrent renal stones with flank pain and haematuria (1). In two patients, signs of renal involvement preceded the onset of sicca syndrome. Renal biopsies from nine patients showed tubulo‐interstitial nephritis in six and glomerular disease in three. Patients with renal involvement had a significantly shorter disease duration compared with patients without renal abnormalities.

Conclusions. Kidney involvement is a frequent extraglandular manifestation of primary Sjögren's syndrome. It is rarely overt and may precede the onset of subjective sicca syndrome.

Keywords: defective urine concentrating capacity; glomerulonephritis; renal tubular acidosis; Sjögren's syndrome; tubulointerstitial nephritis

Journal Article.  5499 words.  Illustrated.

Subjects: Nephrology

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