title: A multicentre study of 95 biopsy-proven cases of renal disease in primary Sjögren's syndrome.
abstract: 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.
  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.
  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.
  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.
authors.affiliation: Department of Nephrology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Sud, Le Kremlin-Bicêtre.
authors.forename: Magali
authors.initials: M
authors.lastname: Jasiek
authors.affiliation: Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Karras
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris.
authors.forename: Véronique
authors.initials: V
authors.lastname: Le Guern
authors.affiliation: Department of Epidemiology and Statistics, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre.
authors.forename: Evguenia
authors.initials: E
authors.lastname: Krastinova
authors.affiliation: Department of Nephrology, Centre Hospitalier, Boulogne-Sur-Mer.
authors.forename: Rafik
authors.initials: R
authors.lastname: Mesbah
authors.affiliation: Department of Nephrology and Organ Transplantation, Hôpital Rangueil, Toulouse.
authors.forename: Stanislas
authors.initials: S
authors.lastname: Faguer
authors.affiliation: Department of Nephrology, Hôpital Conception, Marseille.
authors.forename: Noémie
authors.initials: N
authors.lastname: Jourde-Chiche
authors.affiliation: Department of Internal Medicine, Hôpital Dupuytren, Limoges.
authors.forename: Anne-Laure
authors.initials: AL
authors.lastname: Fauchais
authors.affiliation: Department of Internal Medicine, Hôpital Européen, Marseille.
authors.forename: Laurent
authors.initials: L
authors.lastname: Chiche
authors.affiliation: Department of Rheumatology, Centre Hospitalier, Le Mans.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Dernis
authors.affiliation: Department of Internal Medicine, Hôpital Purpuan, Toulouse.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Moulis
authors.affiliation: Department of Internal Medicine, Centre Hospitalier, Béziers.
authors.forename: Jean-Baptiste
authors.initials: JB
authors.lastname: Fraison
authors.affiliation: Department of Internal Medicine, Hôpital Haut Lévêque, Pessac.
authors.forename: Estibaliz
authors.initials: E
authors.lastname: Lazaro
authors.affiliation: Department of Internal Medicine, CHU Saint-Étienne, Saint-Étienne.
authors.forename: Perrine
authors.initials: P
authors.lastname: Jullien
authors.affiliation: Department of Internal Medicine, Hôpital Claude Huriez, Université Lille 2, Lille.
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.affiliation: Department of Internal Medicine, Hôpital Saint-Eloi, Montpellier.
authors.forename: Alain
authors.initials: A
authors.lastname: Le Quellec
authors.affiliation: Department of Nephrology, Hôpital Henri Mondor, Créteil.
authors.forename: Philippe
authors.initials: P
authors.lastname: Rémy
authors.affiliation: Department of Nephrology, Hôpital Necker.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Hummel
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.affiliation: Department of Nephrology, Hôpital Tenon, Paris.
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
authors.affiliation: Department of Nephrology, Centre Hospitalier, Valenciennes.
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.affiliation: Unité d'Analyse d'Image Quantitative, Institut Pasteur, Paris.
authors.forename: Vannary
authors.initials: V
authors.lastname: Meas-Yedid
authors.affiliation: Department of Pathology, CHU Amiens, Amiens.
authors.forename: Carole
authors.initials: C
authors.lastname: Cordonnier
authors.affiliation: Department of Pathology, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre.
authors.forename: Sophie
authors.initials: S
authors.lastname: Ferlicot
authors.affiliation: Department of Pathology, Hôpital Conception, Marseille.
authors.forename: Laurent
authors.initials: L
authors.lastname: Daniel
authors.affiliation: Department of Rheumatology, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre.|Center of Immunology of Viral and Autoimmune Diseases (IMVA), INSRM U1184, Le Kremlin-Bicêtre, France.
authors.forename: Raphaele
authors.initials: R
authors.lastname: Seror
authors.affiliation: Department of Rheumatology, Hôpital Bicêtre, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre.|Center of Immunology of Viral and Autoimmune Diseases (IMVA), INSRM U1184, Le Kremlin-Bicêtre, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.affiliation: Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP.
authors.forename: Eric
authors.initials: E
authors.lastname: Thervet
authors.affiliation: Department of Nephrology, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Université Paris-Sud, Le Kremlin-Bicêtre.
authors.forename: Hélène
authors.initials: H
authors.lastname: François
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000276:immunology; D001402:B-Lymphocytes / Q000473:pathology; D001706:Biopsy; D003450:Cryoglobulins; D005260:Female; D005602:France; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D010950:Plasma Cells / Q000473:pathology; D051437:Renal Insufficiency / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology / Q000473:pathology; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use; D012859:Sjogren's Syndrome / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D013601:T-Lymphocytes / Q000473:pathology; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D003450:Cryoglobulins; D007166:Immunosuppressive Agents; C035356:SS-A antibodies; C035357:SS-B antibodies; D000069283:Rituximab
keywords: cryoglobulinaemia; primary Sjögren’s syndrome; tubulointerstitial nephritis