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Published on: October 13, 2023
Renal involvement in primary Sjögren's syndrome
Rhys Evans1, Anselm Zdebik1, Coziana Ciurtin2
1UCL Centre for Nephrology, UCL Medical School, Rowland Hill Street and.
Systemic sclerosis (SS) is a common systemic disease affecting epithelial tissue and potentially causing kidney damage through tubulointerstitial nephritis or glomerulopathy. This review covers renal SS prevalence, presentation, pathogenesis, and treatment.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Systemic sclerosis (SS) is a prevalent, underdiagnosed systemic autoimmune disease.
- It primarily targets epithelial tissues but frequently impacts renal function.
- Renal involvement in SS can manifest as tubulointerstitial nephritis or glomerulopathy.
Purpose of the Study:
- To review the prevalence of renal involvement in systemic sclerosis.
- To discuss the clinical presentation and pathogenesis of kidney disease in SS.
- To outline current treatment strategies for renal SS.
Main Methods:
- Literature review of studies on renal manifestations of systemic sclerosis.
- Analysis of disease mechanisms involving B cells, T cells, and Th17 subtypes.
- Synthesis of information on clinical features, diagnosis, and management.
Main Results:
- Renal complications in SS present with diverse clinical features, ranging from overt to occult.
- Pathogenesis involves both direct epithelial damage and immune complex deposition.
- Th17 cells play a notable role in the immune-mediated epithelial disease.
Conclusions:
- Renal involvement is a significant complication of systemic sclerosis.
- Understanding the pathogenesis is crucial for developing targeted therapies.
- Comprehensive management strategies are needed to address renal SS effectively.
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