title: Light chain deposition disease without glomerular proteinuria: a diagnostic challenge for the nephrologist.
abstract: BACKGROUND
  Renal involvement in light chain (LC) deposition disease (LCDD) is typically characterized by nodular glomerulosclerosis and nephrotic range proteinuria. Rare cases of LCDD without glomerular symptoms have been reported, but clinical and pathological characteristics of this entity remain poorly described.
  
  
  METHODS
  This multi-centre retrospective study included 14 patients with biopsy-proven renal LCDD and proteinuria <0.5 g/day at diagnosis.
  
  
  RESULTS
  Baseline median serum creatinine was 281 (136-594) μmol/L, with a glomerular filtration rate of 20 (6-48) mL/min/1.73 m(2). A serum monoclonal immunoglobulin was detected in 12 cases and LC proteinuria only in 7, always of kappa isotype. Monoclonal gammopathy of undetermined significance/indolent multiple myeloma (MM) was diagnosed in nine cases, symptomatic MM in three cases. Hypertension was almost constant (10 of 14). Immunofluorescence studies of kidney biopsies showed linear kappa LC deposition along tubular basement membranes in all cases, with linear glomerular and vascular LC deposits in 11 and 10 patients, respectively. By light microscopy, tubulo-interstitial lesions were prominent in all patients and focal nodular glomerulosclerosis was only observed in two cases. Identification of LCDD led to initiation of chemotherapy in 12 cases. After a median follow-up of 25.5 months, five patients died and four progressed to end-stage renal disease. Renal response occurred in five of the eight patients who achieved sustained haematological response.
  
  
  CONCLUSIONS
  LCDD can cause severe renal dysfunction, despite the absence of glomerular symptoms. Early identification of the disease and introduction of a chemotherapy targeting the underlying plasma cell disorder may preserve long-term renal prognosis.
authors.affiliation: Néphrologie, Hôpital Européen Georges Pompidou, Paris, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Sicard
authors.affiliation: Néphrologie, Hôpital Européen Georges Pompidou, Paris, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Karras
authors.affiliation: Anatomo-Pathologie Rénale, CHU, Poitiers, France Centre de référence amylose AL et autres maladies de dépôt d'immunoglobulines monoclonales.
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Goujon
authors.affiliation: Centre de référence amylose AL et autres maladies de dépôt d'immunoglobulines monoclonales Centre de référence amylose AL et autres maladies de dépôt d'immunoglobulines monoclonales.
authors.forename: Christophe
authors.initials: C
authors.lastname: Sirac
authors.affiliation: Centre de référence amylose AL et autres maladies de dépôt d'immunoglobulines monoclonales CNRS UMR 7276, Limoges, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Bender
authors.affiliation: Néphrologie, CH, Valenciennes, France.
authors.forename: Delphine
authors.initials: D
authors.lastname: Labatut
authors.affiliation: Anatomo-Pathologie, Hôpital Tenon, Paris, France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Callard
authors.affiliation: Hématologie, Hôpital André Mignot, Versailles, France.
authors.forename: Clémentine
authors.initials: C
authors.lastname: Sarkozy
authors.affiliation: Néphrologie, CHU, Limoges, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Essig
authors.affiliation: Néphrologie, CH, Valenciennes, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.affiliation: Néphrologie, CHU, Lille, France.
authors.forename: François
authors.initials: F
authors.lastname: Provot
authors.affiliation: Néphrologie, CH, Bourges, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Nony
authors.affiliation: Anatomo-Pathologie, HEGP, Paris, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Nochy
authors.affiliation: Néphrologie, Hôpital Tenon, Paris, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
authors.affiliation: Centre de référence amylose AL et autres maladies de dépôt d'immunoglobulines monoclonales Néphrologie, CHU, Poitiers, France.
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.affiliation: Centre de référence amylose AL et autres maladies de dépôt d'immunoglobulines monoclonales Néphrologie, CHU, Poitiers, France.
authors.forename: Guy
authors.initials: G
authors.lastname: Touchard
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000595:Amino Acid Sequence; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains; D007674:Kidney Diseases / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D007678:Kidney Glomerulus / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D009101:Multiple Myeloma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D010265:Paraproteinemias / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D011379:Prognosis; D011507:Proteinuria / Q000175:diagnosis*; D012189:Retrospective Studies; D017386:Sequence Homology, Amino Acid; D015996:Survival Rate
chemical_list: D007147:Immunoglobulin Light Chains
keywords: light chain deposition disease; monoclonal gammopathy; myeloma; proteinuria; renal failure 