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Renal involvement in primary Sjögren's syndrome: a clinicopathologic study
Saugar Maripuri1, Joseph P Grande, Thomas G Osborn
1Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Kidney biopsies in primary Sjögren's syndrome (pSS) reveal tubulointerstitial nephritis as the main finding. Early treatment with immunosuppressants helps preserve kidney function in most affected patients.
Area of Science:
- Nephrology
- Rheumatology
- Pathology
Background:
- Primary Sjögren's syndrome (pSS) can affect the kidneys, leading to renal impairment.
- Kidney biopsy (KB) is crucial for diagnosing renal pathology in pSS patients with kidney dysfunction.
Purpose of the Study:
- To report renal pathology and clinical outcomes in patients with pSS who underwent KB due to renal impairment.
- To evaluate the impact of treatment on renal function in these patients.
Main Methods:
- A retrospective review of 24 patients with pSS who underwent KB over 40 years.
- Cases were analyzed by renal pathologists, nephrologists, and rheumatologists, noting presentation, lab findings, pathology, treatment, and response.
Main Results:
- Tubulointerstitial nephritis (TIN) was the primary lesion in 71% of patients, with chronic TIN being most common (65%).
- Eighty-three percent of patients were initially treated with corticosteroids; 14 of 16 followed for over a year maintained or improved renal function.
- No patients presenting with stage IV chronic kidney disease progressed to stage V with treatment.
Conclusions:
- Chronic TIN is the predominant KB finding in renal involvement from pSS, alongside diverse glomerular lesions.
- KB is recommended for evaluating kidney dysfunction in pSS and may serve as a classification criterion.
- Treatment with glucocorticoids or immunosuppressants appears to slow renal disease progression.
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