SS is a prevalent and underdiagnosed systemic disease that primarily affects epithelial tissue. It may affect renal function either as epithelial disease causing tubulointerstitial nephritis or as an immune complex-mediated glomerulopathy. These lesions may cause a variety of clinical features, both overt and occult. The epithelial disease is mediated by B and T cells, notably the Th17 subtype. We review the prevalence of renal SS, its presentation, likely pathogenesis and treatment.
Keywords: B cells; Fanconi syndrome; Sjögren’s syndrome; Th17 cells; autoantibodies; autoimmune epithelialitis; distal renal tubular acidosis; hypocomplementaemia; tubulointerstitial nephritis; vasculitis.
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