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.
MeSH terms
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Acidosis, Renal Tubular / etiology
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Adrenal Cortex Hormones / therapeutic use
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Aged
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Antibodies, Antinuclear / immunology
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Autoantibodies / immunology*
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Autoantigens / immunology
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Autoimmune Diseases / complications*
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Autoimmune Diseases / immunology
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Autoimmune Diseases / pathology
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Biopsy
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Bone Diseases, Metabolic / etiology
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Calcitriol / therapeutic use
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Fanconi Syndrome / etiology*
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Fanconi Syndrome / immunology
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Fanconi Syndrome / pathology
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Female
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Humans
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Ketoglutarate Dehydrogenase Complex / immunology
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Kidney / pathology
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Kidney Failure, Chronic / etiology
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Liver / pathology
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Liver Cirrhosis, Biliary / complications*
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Liver Cirrhosis, Biliary / immunology
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Liver Cirrhosis, Biliary / pathology
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Middle Aged
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Mitochondria / immunology
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Mitochondria, Liver / enzymology
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Nephritis, Interstitial / etiology*
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Nephritis, Interstitial / immunology
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Nephritis, Interstitial / pathology
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Phosphates / blood
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Pyruvate Dehydrogenase Complex / immunology
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Ursodeoxycholic Acid / therapeutic use
Substances
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Adrenal Cortex Hormones
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Antibodies, Antinuclear
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Autoantibodies
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Autoantigens
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Phosphates
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Pyruvate Dehydrogenase Complex
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Ursodeoxycholic Acid
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Ketoglutarate Dehydrogenase Complex
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Calcitriol