title: Non-Randall proliferative glomerulonephritis with humps and monotypic IgG deposits in primary Sjögren's syndrome: a first case report.
abstract: Renal involvement is frequent in patients suffering from primary Sjögren's syndrome (pSS). Tubulointerstitial infiltration is the most common renal lesion, while glomerular involvement is rare. We report the case of a 50-year-old woman with pSS who developed renal failure due to an unusual proliferative glomerulonephritis with humps and monotypic IgG1-kappa deposits. Searches for cryoglobulinaemia, anti-double-stranded DNA and anti-neutrophil cytoplasmic antibodies were negative. Serum protein electrophoresis and immunofixation revealed no monoclonal immunoglobulin. Extensive work-up excluded associated infectious, collagen or lymphoproliferative disease. This case adds to the spectrum of pSS-related glomerular disease which is reviewed in depth.
authors.affiliation: AP-HP, Hôpital Tenon , Service de Néphrologie et Dialyses, F-75020, Paris , France.
authors.forename: Karine
authors.initials: K
authors.lastname: Dahan
authors.affiliation: AP-HP, Hôpital Tenon , Service de Néphrologie et Dialyses, F-75020, Paris , France ; Hôpital de Chartres , Service de Néphrologie, Chartres , France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Albert
authors.affiliation: AP-HP, Hôpital Tenon , Service de Néphrologie et Dialyses, F-75020, Paris , France.
authors.forename: Jean-Benoît
authors.initials: JB
authors.lastname: Arlet
authors.affiliation: AP-HP, Hôpital Tenon , Service d'Anatomie pathologique, F-75020, Paris , France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Callard
authors.affiliation: AP-HP, Hôpital Tenon , Service de Néphrologie et Dialyses, F-75020, Paris , France ; UPMC Univ Paris 06, Paris , France ; INSERM , UMR_S702, F-75020, Paris , France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
keywords: Sjögren’s syndrome; crescentic glomerulonephritis; renal involvement