title: Tubulointerstitial nephritis and Fanconi syndrome in primary biliary cirrhosis.
abstract: Primary biliary cirrhosis is a chronic cholestatic liver disease of unknown cause that predominantly affects middle-aged women. Distal tubular acidosis is the main renal complication of primary biliary cirrhosis. Tubulointerstitial nephritis and Fanconi syndrome have been reported more rarely. We report on 2 patients with primary biliary cirrhosis who presented with tubulointerstitial nephritis and Fanconi syndrome and review similar cases published previously. Serum from 1 patient exerted an inhibitory effect on pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, 2 mitochondrial enzymes that are the main targets of antimitochondrial antibodies in primary biliary cirrhosis. Antimitochondrial antibodies may have a role in the genesis of tubulointerstitial nephritis and Fanconi syndrome, 2 typical renal features of mitochondrial cytopathies. Tubulointerstitial nephritis and Fanconi syndrome have to be added to the spectrum of renal diseases associated with primary biliary cirrhosis.
authors.affiliation: Service d'Hépatologie AP-HP, Hôpital Necker, Paris, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Lino
authors.forename: Raynald
authors.initials: R
authors.lastname: Binaut
authors.forename: Laure-Hélène
authors.initials: LH
authors.lastname: Noël
authors.forename: Natacha
authors.initials: N
authors.lastname: Patey
authors.forename: Pierre
authors.initials: P
authors.lastname: Rustin
authors.forename: Laurent
authors.initials: L
authors.lastname: Daniel
authors.forename: Jeanne
authors.initials: J
authors.lastname: Serpaggi
authors.forename: Anne
authors.initials: A
authors.lastname: Varaut
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.forename: Bertrand
authors.initials: B
authors.lastname: Knebelmann
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Grünfeld
authors.forename: Fadi
authors.initials: F
authors.lastname: Fakhouri
mesh_terms: D000141:Acidosis, Renal Tubular / Q000209:etiology; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D000974:Antibodies, Antinuclear / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology / Q000473:pathology; D001706:Biopsy; D001851:Bone Diseases, Metabolic / Q000209:etiology; D002117:Calcitriol / Q000627:therapeutic use; D005198:Fanconi Syndrome / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007655:Ketoglutarate Dehydrogenase Complex / Q000276:immunology; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D008930:Mitochondria, Liver / Q000201:enzymology; D009395:Nephritis, Interstitial / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D010710:Phosphates / Q000097:blood; D011768:Pyruvate Dehydrogenase Complex / Q000276:immunology; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D001324:Autoantigens; D010710:Phosphates; D011768:Pyruvate Dehydrogenase Complex; D014580:Ursodeoxycholic Acid; D007655:Ketoglutarate Dehydrogenase Complex; D002117:Calcitriol