PMID: 15224808Jul 1, 2004Paper

Proximal tubular dysfunction in primary Sjögren's syndrome: a clinicopathological study of 2 cases

Clinical Nephrology
F BridouxP Vanhille

Abstract

Tubulointerstitial nephritis is the most common renal complication in primary Sjögren's syndrome (SS). It is usually associated with symptoms of distal tubular dysfunction, type I (distal) renal tubular acidosis (RTA) and nephrogenic diabetes insipidus. Proximal tubular abnormalities are considered to be less frequent, and Fanconi's syndrome has been only exceptionally reported in patients with SS. We describe 2 patients with primary SS, characterized by xerostomia, dry eyes, extensive lymphocytic infiltrate on salivary gland biopsy, positive tests for anti-SSA/SSB antibodies and/or antinuclear antibodies, who presented in renal failure with proteinuria, microscopic hematuria and type I RTA. Further studies revealed proximal tubular dysfunction, including renal glucosuria, generalized aminoaciduria, phosphaturia, uricosuria, together with proximal (type II) RTA in 1 case. Neither of these patients had Bence Jones proteinuria or monoclonal gammopathy. Kidney biopsy showed focal proximal tubulitis, associated with proximal tubular cell atrophy and dedifferentiation, and diffuse interstitial nephritis with fibrosis. No significant glomerular or peritubular deposits of immunoglobulin light or heavy chain were observed. These findin...Continue Reading

Citations

Nov 10, 2006·Rheumatology International·Yi-Sun YangChien-Ning Huang
Oct 11, 2011·Modern Rheumatology·Rajesh RajputOmpal Saini
Aug 12, 2014·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Gulperi CelikLevent Kebapcilar
Mar 12, 2016·The Journal of International Medical Research·Mingmin Shi, Lei Chen
Nov 17, 2015·Nature Reviews. Nephrology·Hélène François, Xavier Mariette
Apr 24, 2010·Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases·Chih-Chiang WangShih-Hua Lin
Jan 15, 2015·The Open Rheumatology Journal·Pulukool SandhyaNihal Thomas
May 10, 2008·Scandinavian Journal of Rheumatology·I KaufmanD Paran
Nov 20, 2020·Néphrologie & thérapeutique·Hélène François, Xavier Mariette

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