Sjögrenn and TIN

Renal disease in Sjögren syndrome (SS) is common; its prevalence in some series being as high as 42 %. Rarely, SS can cause a glomerular lesion with decreased excretory renal function and proteinuria. The lesion itself is typically a membranoproliferative glomerulonephritis (MPGN), but can present with a number of other glomerular lesions (e.g. membranous nephropathy). This is due to immune complex deposition associated with B-cell expansion, cryoglobulinaemia and lymphoma. However, epithelial inflammation in SS typically causes tubulointerstitial nephritis, the commonest renal lesion in SS. Although this may cause renal impairment, it also causes renal tubular lesions which may be more difficult to diagnose (Evans RD, et al. Tubulointerstitial nephritis in primary Sjögren syndrome: clinical manifestations and response to treatment. BMC Musculoskelet Disord. 2016;17:2. [PubMed link]).

Patients may have extraglandular disease involving multiple organs, including the kidneys. Kidney disease in SS presents a diagnostic challenge, as clinical symptoms are often insidious and can precede sicca symptoms. SS affects the kidney through lymphocytic infiltration of renal tubules or immune complex deposition, leading to an array of clinical features. Tubulointerstitial nephritis is the most common histological pattern of kidney disease. Other tubular injuries include renal tubular acidosis with hypokalaemia, Fanconi's syndrome and diabetes insipidus. Glomerular disease is less common and typically involves an immune complex-mediated process. Optimal treatment for kidney diseases in SS is not established, and treatment is guided by the pattern of disease. For tubulointerstitial nephritis, management involves electrolyte imbalance correction and the use of immunosuppression, including steroids (Aiyegbusi O, et al. Renal Disease in Primary Sjögren's Syndrome. Rheumatol Ther. 2021;8(1):63-80. [PubMed link]).

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