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Renal disease in Sjögren's syndrome

INTRODUCTION

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 nonexocrine organs, including the kidneys, producing an interstitial nephritis and defects in tubular function. (See "Clinical manifestations of Sjögren's syndrome: Extraglandular disease and prognosis".)

The reported prevalence of renal involvement has varied widely, ranging from 2 to 67 percent [1-3]. This variability is in part due to different definitions of kidney involvement.

INTERSTITIAL NEPHRITIS

The interstitial nephritis in Sjögren's syndrome is characterized histologically by an interstitial infiltrate that can invade and damage the tubules [3-5]. In some cases, granuloma formation is seen and there may be a concurrent uveitis, suggesting the possible presence of sarcoidosis or the TINU syndrome [6]. (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 (see 'Glomerular disease' below [7].

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, type 1 (distal) renal tubular acidosis (RTA), nephrogenic diabetes insipidus (tubular resistance to antidiuretic hormone), and hypokalemia [5,8-14].
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References Top
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