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Renal disease in Sjögren's syndrome

Jai Radhakrishnan, MD, MS
Section Editor
Gary C Curhan, MD, ScD
Deputy Editor
Alice M Sheridan, MD


Sjögren's syndrome is typically associated with a lymphocytic and plasmacytic infiltrate in the salivary, parotid, and lacrimal glands, leading to a sicca syndrome. This immune process can also affect non-exocrine organs, including the kidneys, producing an interstitial nephritis and defects in tubular function. (See "Clinical manifestations of Sjögren's syndrome: Extraglandular disease".)

The reported prevalence of renal involvement has varied widely, ranging from 2 to 67 percent [1-3]. This variability is due in part to different definitions of kidney involvement. In a renal biopsy series of 25 patients, Sjögren’s syndrome preceded renal manifestations by a median of 5.5 years [4]. Twenty-two patients were female, and the median age at the time of biopsy was 55 years. Thirteen patients had proteinuria >1 g, and estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2 was seen in 21 patients; more than half the patients had eGFR <30 mL/min/1.73 m2. In another series, renal involvement occurred in 4.9 percent of 715 patients with Sjögren’s syndrome, with glomerular involvement occurring more frequently than tubulointerstitial disease; 37 percent had interstitial nephritis alone, 49 percent had glomerulonephritis alone, and 14 percent had both. The prognosis appears to be worse in patients with predominantly glomerular involvement, with lower survival rates and higher incidence of lymphoma, compared with patients with predominantly tubulointerstitial involvement [5].


Chronic tubulointerstitial nephritis is the most common renal manifestation of Sjögren’s syndrome [4,6]. The interstitial nephritis in Sjögren's syndrome is characterized histologically by an interstitial infiltrate that can invade and damage the tubules [3,7,8]. In some cases, granuloma formation is seen, and there may be a concurrent uveitis, suggesting the possible presence of sarcoidosis or the tubulointerstitial nephritis and uveitis (TINU syndrome) [6,9]. (See "Renal disease in sarcoidosis" and "Tubulointerstitial nephritis and uveitis (TINU syndrome)".)

More chronic disease is associated with tubular atrophy and interstitial fibrosis. The glomeruli are usually normal, although an immune complex-mediated glomerulonephritis has rarely been described [10]. (See 'Glomerular disease' below.)

The clinical manifestations of the interstitial nephritis include a variable but generally mild elevation in the plasma creatinine concentration, a relatively benign urinalysis, and abnormalities in tubular function, including the Fanconi syndrome, distal (type 1) renal tubular acidosis (RTA), nephrogenic diabetes insipidus (tubular resistance to antidiuretic hormone), and hypokalemia [8,11-17].


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Literature review current through: Sep 2016. | This topic last updated: Jul 13, 2016.
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