Abstract
Primary Sjogren's syndrome (pSS) is an autoimmune disorder in which lymphocytic infiltration leads to lacrimal and salivary glands dysfunction, which results in symptoms of dryness (xerophthalmia and xerostomia). Extraglandular features are common and may affect several organs. Renal involvement has long been known as one of the systemic complications of pSS. The most classical lesion observed in pSS is tubulointerstitial nephritis (TIN) and less frequently membranoproliferative glomerulonephritis (MPGN), which is related to cryoglobulinemia. In some cases, renal biopsy is necessary for the definitive diagnosis of kidney involvement. Patients may present with proximal renal tubular acidosis, distal renal tubular acidosis and chronic kidney disease. Response to treatment is usually favorable. However, occasionally severe and rarely lethal outcomes have been described. Recently, several case series and cross-sectional studies have been published which investigated the factors associated with renal involvement in pSS and the most accurate screening tests for early detection. The presence of xerophthalmia, anti-SSA and rheumatoid factor positivity, low C3 levels and other features have all shown either positive or inverse associations with the development of renal complications. Serum creatinine, alpha-1-microglobulin, cystatin-C have been evaluated as early detection biomarkers with variable accuracy. More advanced techniques may be necessary to confirm proximal and distal renal tubular acidosis, along with nephrogenic diabetes insipidus. The aim of the current paper is to summarize and critically examine these findings in order to provide updated guidance on serum biomarkers and further testing for kidney involvement in pSS.
Highlights
Sjögren’s syndrome (SS) is a systemic autoimmune disease which primarily causes dysfunction of exocrine glands
This review aims to shed light on the most recent developments in the field, with a special focus on biomarkers and diagnostic testing necessary to elicit subclinical renal damage (5, 6)
A characteristic finding of tubulointerstitial nephritis (TIN) in Primary Sjogren’s syndrome (pSS) is the presence of distal renal tubular acidosis (RTA), which presents with an elevated urinary pH (>5.5) in the setting of an hyperchloremic metabolic acidosis (6, 16)
Summary
Sjögren’s syndrome (SS) is a systemic autoimmune disease which primarily causes dysfunction of exocrine glands. This leads to dryness of the ocular and oral mucosa (xerophthalmia and xerostomia), along with possible involvement of the pharynx, larynx, and vagina (1). SS can be complicated by severe manifestations such as multiorgan involvement and hematologic malignancies (2). A number of genetic and environmental factors may intertwine in the etiology of SS. The disease predominantly affects females (in a 9/1 ratio) in their middle age, but it can affect different populations (1)
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