Renal Involvement in Primary Sjögren’s Syndrome: A Multicenter French Study of 95 Biopsy Proven Cases

Benjamin Terrier¹, Magali Jasiek², Hélène Francois³, Rafik Mesbah⁴, Laurent Chiche⁵, Anne-Laure Fauchais⁶, Guillaume Moulis⁷, Guillaume Le Guenno⁸, Estibaliz Lazaro⁹, Nathalie Costedoat-Chalumeau¹⁰, Raphaèle Seror¹¹, Luc Mouthon¹, Loïc Guillevin¹, Xavier Mariette¹², Eric Thervet¹³, Alexandre Karras¹⁴ and Véronique Le Guern¹⁵, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, ²Nephrology, Bicêtre Hospital, University Paris Sud, France, Paris, France, ³1Nephrology, Bicêtre Hospital, University Paris Sud, France, PARIS, France, ⁴Nephrology, Hôpital Boulogne sur Mer, France, Boulogne Sur Mer, France, ⁵147 Boulevard Baille, CHU Marseille, Marseille, France, ⁶Department of Internal Medicine A, Dupuytren Hospital, Limoges University Hospital, Limoges, France, ⁷Internal Medicine department, Toulouse, Toulouse, France, ⁸Internal Medicine department, Clermont-Ferrand, France, ⁹Hôpital Haut-Lévêque, Pessac, France, ¹⁰Internal medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, ¹¹Rheumatology, Université Paris Sud, Le Kremlin Bicêtre, France, ¹²rheumatology, Université Paris-Sud, Le Kremlin Bicêtre, France, ¹³Nephrology, Hopital Européen Georges Pompidou, APHP, PARIS, France, ¹⁴Nephrology, Hôpital Européen Georges Pompidou, APHP, Paris, France, ¹⁵Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: Kidney and Sjogren's syndrome

Session Information

Title: Sjogren's Syndrome: Clinical Science

Session Type: Abstract Submissions (ACR)

Background/Purpose

Renal involvement is reported in 5 to 25% of patients with primary Sjögren’s syndrome (pSS). However, data on pSS-related nephropathy with renal biopsy (RB) remain scarce. This observational study was undertaken to describe the clinical and histopathological characteristics of pSS-related nephropathies and their outcomes.

Methods

We conducted a French multicenter and transdisciplinary retrospective study on pSS patients with RB-proven nephropathies. Inclusion criteria were patients diagnosed with pSS based on American-European Consensus Group (AECG) criteria or study-specific enlarged AECG criteria (presence of ≥3 of 4 AECG items), and who underwent a RB.

Results

Ninety-five patients were included (sex ratio F/M 9/1, mean age 49 years), 84% and 16% fulfilling AECG criteria and enlarged AECG criteria respectively. pSS was isolated in 88% or associated with other organ-specific autoimmune disorders (12%), including primary biliary cirrhosis, autoimmune hepatitis and thyroiditis. Renal disease was diagnosed prior to pSS for 20%, with a median interval of 17 months. For 41%, the 2 conditions were diagnosed simultaneously and, for 39%, after pSS, with a median interval of 36 months. Renal manifestations consisted of renal failure (86%), glomerular-range proteinuria (26%), electrolyte disturbances alone (18%), lithiasis (10%) and/or nephrocalcinosis (5%). RB exhibited acute or chronic tubulointerstitial nephritis (77%) with numerous plasma-cell infiltrates (69%). Glomerular lesions were found in 23%, associated with interstitial infiltrates in most patients. Glomerular disease was mainly cryoglobulinemic glomerulonephritis, focal and segmental glomerulosclerosis or membranous nephropathy. Eighty patients (84%) received corticosteroids (CS). In 21 patients (22%), standard immunosuppressive drugs (n=8) and/or rituximab (n=18) was added on top of CS in a first or a second line of treatment. Baseline mean estimated glomerular filtration rate (eGFR) was 40 mL/min/1,73m2. After a median follow-up of 53 months, eGFR improved significantly (47 mL/min/1,73m2, P=0.001), and only 3 patients progressed to end-stage renal disease. Multivariate analyses found age and tubulointerstitial lesions as significantly associated factors with poor renal prognosis, even though overall outcome was good.

Conclusion

In pSS, the main renal lesion is tubulointerstitial nephropathy associated with significant and frequent renal dysfunction. Systemic treatments can significantly improve renal function. Analysis of renal prognosis associated with the different therapeutic strategies is ongoing.

Disclosure:

B. Terrier,
None;

M. Jasiek,
None;

H. Francois,
None;

R. Mesbah,
None;

L. Chiche,
None;

A. L. Fauchais,
None;

G. Moulis,
None;

G. Le Guenno,
None;

E. Lazaro,
None;

N. Costedoat-Chalumeau,
None;

R. Seror,
None;

L. Mouthon,
None;

L. Guillevin,
None;

X. Mariette,
None;

E. Thervet,
None;

A. Karras,
None;

V. Le Guern,
None.

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