XXXX in primary Sj  gren syndrome: clinical manifestations and response to treatment.

@entity1 @entity8 @entity404 @entity6051 @entity4434 @entity1986 @entity230 @entity1011 @entity631 @entity792 @entity288
BACKGROUND: Primary Sj  gren syndrome (pSS) is a common @entity792 which primarily affects epithelial tissue, often including the kidney causing either @entity1986 ( @entity1986 ) or more rarely, an immune complex related @entity631 . METHODS: We describe the clinical, biochemical and histological characteristics of 12 @entity1 with pSS related @entity1986 and their response to treatment with antiproliferative agents. All 12 @entity1 were investigated and treated at the UCL Centre for Nephrology in London. RESULTS: All @entity1 had @entity1986 demonstrated via needle biopsy; immunophenotyping showed that the interstitial infiltrate was predominantly a @entity404 + T-cell infiltrate. Urinary acidification testing demonstrated @entity8 in 8 @entity1 . Proximal @entity4434 was present in 5 @entity1 . All but 1 @entity1 were treated with antiproliferative agents and most also with a reducing course of @entity230 . In the treated @entity1 , there was a significant improvement in the serum @entity1011 and measured GFR. CONCLUSION: @entity1 with pSS @entity1986 have significant @entity8 and other functional tubular defects. There is a mononuclear lymphocytic infiltrate on renal biopsy and this appears to be mainly a @entity404 + T-cell infiltrate. Treatment with @entity6051 (and @entity288 ) improves the renal function in @entity1 with pSS @entity1986 .
