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Rheumatology (Oxford). 2017 Mar 1;56(3):362-370. doi: 10.1093/rheumatology/kew376.

A multicentre study of 95 biopsy-proven cases of renal disease in primary Sjögren's syndrome.

Author information

1
Department of Nephrology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Sud, Le Kremlin-Bicêtre.
2
Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP.
3
Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris.
4
Department of Epidemiology and Statistics, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre.
5
Department of Nephrology, Centre Hospitalier, Boulogne-Sur-Mer.
6
Department of Nephrology and Organ Transplantation, Hôpital Rangueil, Toulouse.
7
Department of Nephrology, Hôpital Conception, Marseille.
8
Department of Internal Medicine, Hôpital Dupuytren, Limoges.
9
Department of Internal Medicine, Hôpital Européen, Marseille.
10
Department of Rheumatology, Centre Hospitalier, Le Mans.
11
Department of Internal Medicine, Hôpital Purpuan, Toulouse.
12
Department of Internal Medicine, Centre Hospitalier, Béziers.
13
Department of Internal Medicine, Hôpital Haut Lévêque, Pessac.
14
Department of Internal Medicine, CHU Saint-Étienne, Saint-Étienne.
15
Department of Internal Medicine, Hôpital Claude Huriez, Université Lille 2, Lille.
16
Department of Internal Medicine, Hôpital Saint-Eloi, Montpellier.
17
Department of Nephrology, Hôpital Henri Mondor, Créteil.
18
Department of Nephrology, Hôpital Necker.
19
Department of Nephrology, Hôpital Tenon, Paris.
20
Department of Nephrology, Centre Hospitalier, Valenciennes.
21
Unité d'Analyse d'Image Quantitative, Institut Pasteur, Paris.
22
Department of Pathology, CHU Amiens, Amiens.
23
Department of Pathology, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre.
24
Department of Pathology, Hôpital Conception, Marseille.
25
Department of Rheumatology, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre.
26
Center of Immunology of Viral and Autoimmune Diseases (IMVA), INSRM U1184, Le Kremlin-Bicêtre, France.

Abstract

Objective.:

Renal involvement is a rare event during primary SS (pSS). We aimed to describe the clinico-biological and histopathological characteristics of pSS-related nephropathy and its response to treatment.

Methods.:

We conducted a French nationwide, retrospective, multicentre study including pSS patients fulfilling American-European Consensus Group criteria or enlarged American-European Consensus Group criteria, and with biopsy-proven renal involvement.

Results.:

A total of 95 patients were included (median age 49 years). An estimated glomerular filtration rate (eGFR) of <60 ml/min was found in 82/95 patients (86.3%). Renal biopsy demonstrated tubulointerstitial nephritis (TIN) in 93 patients (97.9%), and frequent (75%) plasma cell infiltrates. Glomerular lesions were found in 22 patients (23.2%), mainly related to cryoglobulin. The presence of anti-SSA (76.8%) and anti-SSB (53.8%) antibodies was particularly frequent among patients with TIN and was associated with a worse renal prognosis. Eighty-one patients (85.3%) were treated, with CSs in 80 (98.8%) and immunosuppressive agents (mostly rituximab) in 21 cases (25.9%). Despite marked interstitial fibrosis at initial biopsy, kidney function improved significantly during the 12-month period following diagnosis (final eGFR 49.9 vs 39.8 ml/min/1.73 m 2 at baseline, P < 0.001). No proven benefit of immunosuppressive agents over steroid therapy alone was found in this study.

Conclusion.:

Renal involvement of pSS is mostly due to TIN with marked T, B and especially plasma cell infiltration. Renal dysfunction is usually isolated but can be severe. Use of CSs can improve the eGFR, but further studies are needed to define the best therapeutic strategy in this disease.

KEYWORDS:

cryoglobulinaemia; primary Sjögren’s syndrome; tubulointerstitial nephritis

PMID:
27940588
DOI:
10.1093/rheumatology/kew376
[Indexed for MEDLINE]
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