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Idiopathic Adulthood Ductopenia

Idiopathic Adulthood Ductopenia The clinical and pathological findings of idiopathic ductopenia were studied in a 30-year-old woman who initially manifested jaundice and pruritus. Serum biochemical tests of liver function indicated severe and progressive cholestasis. Viral hepatitis markers and circulating autoantibodies were absent. The patient had a normal cholangiogram and lacked evidence of inflammatory bowel disease. Histological examination of a liver specimen showed severe cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination. Transplantation resulted in prompt and complete resolution of the jaundice and pruritus. Two types of idiopathic adulthood ductopenia associated with different prognoses are recognized. Patients with type 1 idiopathic adulthood ductopenia are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biopsy specimens. Their clinical course ranges from spontaneous improvement to progression to biliary cirrhosis. In contrast, patients with type 2 idiopathic adulthood ductopenia generally manifest initial symptoms of decompensated biliary cirrhosis, have extensive destruction of the intrahepatic bile ducts on liver biopsy, and frequently require orthotopic liver transplantation.Idiopathic adulthood ductopenia (IAD) is a disease of unknown cause that affects predominantly young adults and is characterized by clinical and biochemical evidence of cholestasis.The hallmark of IAD is the loss of interlobular and septal bile ducts observed on liver biopsy specimens.Since the first description in 1988,several cases of IAD have been reported, yet the pathogenesis remains unknown.In the absence of definite and specific diagnostic criteria, IAD remains a diagnosis of exclusion, to be considered in patients with cholestatic liver disease of obscure cause. Herein we describe a patient with IAD and review the literature to date on this uncommon hepatobiliary disorder.REPORT OF A CASEA 30-year-old Filipino woman who had lived in the United States since 1995 was admitted to our institution in September 1997 with gradually progressive fatigue, jaundice, and severe, generalized pruritus of 4 months' duration. Her medical history consisted only of a cholecystectomy for cholelithiasis and chronic cholecystitis 3 years earlier. She had not had neonatal or childhood jaundice, viral hepatitis, other previous liver disease, alcohol or other drug abuse, or transfusions of blood products. She had no relevant family or social history. The patient denied ingestion during the preceding 12 months of chlorpromazine, prochlorperazine, tolbutamide, or organic arsenicals. On admission, physical examination showed marked scleral icterus and diffuse skin hyperpigmentation. No stigmas of chronic liver disease or xanthelasmas were apparent. Her abdomen was soft and without tenderness. There was no evidence of hepatosplenomegaly, ascites, or prominent collateral venous circulation.Laboratory studies showed normal white and red blood cell counts and a prolonged prothrombin time (21 seconds; reference range, 11-13 seconds). Serum biochemical tests of liver function showed a predominantly cholestatic pattern: total serum bilirubin level, 257 µmol/L (15 mg/dL) (reference range, 0-21 µmol/L [0-1.2 mg/dL]); direct bilirubin level, 136.8 µmol/L (8 mg/dL) (reference range, 0-6.8 µmol/L [0-0.4 mg/dL]); γ-glutamyltransferase level, 250 U/L (reference range, 8-78 U/L); alkaline phosphatase level, 300 U/L (reference range, 34-124 U/L); and elevated aminotransferase levels: aspartate aminotransferase level, 160 U/L (reference range, 8-54 U/L) and alanine aminotransferase level, 210 U/L (reference range, 8-52 U/L). Hepatitis A antibody, hepatitis B surface antigen and antibody, hepatitis B core antibody, hepatitis C antibody, amylase, lipase, α1-antitrypsin, iron, ferritin, and ceruloplasmin levels were all absent or within normal limits. Tests for antinuclear, anti-DNA, antimitochondrial, anti–liver-kidney microsomal type 1, and anti–smooth muscle autoantibodies and rheumatoid factor were all negative as well.Ultrasound of the abdomen demonstrated mild hepatomegaly, without biliary ductal dilatation or parenchymal abnormalities. Endoscopic retrograde cholangiopancreatography showed a normal biliary tract and pancreatic ductal anatomy. Esophagogastroduodenoscopy and colonoscopy were normal. A percutaneous liver biopsy specimen showed a striking absence of interlobular bile ducts, marked cholestasis, portal inflammation, and fibrosis (Figure 1and Figure 2). Biliary cirrhosis secondary to IAD was diagnosed.Figure 1.Pathological analysis of the explanted liver. Macroscopically, the liver exhibited an intense dark-green color, consistent with severe chronic cholestasis. At lower power, absence of interlobular bile ducts in the portal area and severe cholestasis, particularly in zone 1 of the hepatic lobule (periportal), are observed (hematoxylin-eosin, original magnification ×200).Figure 2.Higher-power view of a portal tract confirming the absence of bile ducts and the presence of a mild chronic inflammatory infiltrate that includes lymphocytes, neutrophils, and plasma cells (hematoxylin-eosin, original magnification ×400).The patient's jaundice worsened, with a bilirubin level greater than 342 µmol/L (20 mg/dL). Her pruritus became severe and refractory to the use of cholestyramine resin, ursodiol, rifampin, and even continuous infusions of intravenous naloxone hydrochloride.She developed disabling fatigue. She underwent successful orthotopic liver transplantation, which was followed by prompt disappearance of the pruritus, fatigue, and jaundice. Her postoperative course was uncomplicated, and at follow-up 12 months after transplantation, she was doing well and was free of symptoms, with normal results of liver function studies and no evidence of allograft dysfunction.COMMENTThe case described herein demonstrates the hallmark characteristics of the IAD syndrome, first described by Ludwig et al in 1988.Idiopathic adulthood ductopenia is a chronic cholestatic liver disease of unknown cause characterized by the loss of interlobular and septal bile ducts.Numerous known causes have been implicated in the pathogenesis of intrahepatic biliary destruction. These include developmental biliary atresia (secondary to infection or α1-antitrypsin deficiency),immunologic causes resulting from aberrant expression of HLA class II histocompatibility antigens expressed on the surface of biliary epithelial cells (such as might be the case in primary sclerosing cholangitis, graft vs host disease, sarcoidosis, and primary biliary cirrhosis),infection (cholangitis),ischemia, and chemical injuries (such as injection of scolicidal solution or formaldehyde).Furthermore, a variety of therapeutic drugs have occasionally been associated with bile duct destruction and loss, including chlorpromazine, prochlorperazine, organic arsenicals, and tolbutamide.If these causes are systematically excluded, IAD should be considered as the possible diagnosis. This entity occurs in young to middle-aged adults, without history of infantile cholangiopathy, who manifest cholestatic liver disease. The following criteria are necessary: absence of interlobular bile ducts in at least 50% of small portal tracts on an adequate-sized liver biopsy specimen, normal endoscopic retrograde cholangiopancreatography (thus excluding disorders of medium-size and large biliary ducts, such as primary sclerosing cholangitis), normal colonoscopy (to exclude inflammatory bowel disease–related cholangiopathy), absence of antimitochondrial antibodies (excluding primary biliary cirrhosis), and absence of granulomatous cholangitis, histiocytosis X, neutrophilic suppurative cholangitis, lymphoma, or neoplasm in liver biopsy specimens.Although a distinct cause of IAD has not been determined, several hypotheses have been raised. Some investigators have suggested that IAD may be related to the late expression of a nonsyndromic form of Alagille syndrome (infantile scarcity of interlobular bile ducts), an uncommon disease of childhood.This syndrome is characterized by atresia of interlobular bile ducts and is associated with a combination of the following: chronic cholestasis, triangular face, prominent forehead and hypertelorism, posterior embryotoxon, vertebral arch defects, and peripheral pulmonary hypoplasia.However, IAD shares few characteristics with the nonsyndromic form of Alagille syndromeand lacks the relatively poor prognosis. Half of the patients with the nonsyndromic form of Alagille syndrome develop cirrhosis with portal hypertension and die of liver failure within the first years of life.It is also possible that some patients with IAD have a form of viral cholangitis; however, no specific viral agent has been isolated in affected individuals. Although many patients with IAD have had cholecystectomy, a role for previous bacterial ascending cholangitis has not been proved. The clinical appearance of patients with IAD bears similarities to that of patients with primary sclerosing cholangitis. In fact, pericholangitis is frequently observed surrounding the remaining bile ducts in liver biopsy specimens from patients with IAD. Thus, it is conceivable that IAD could be considered a form of isolated, atypical, or limited variant of small-duct primary sclerosing cholangitisthat nevertheless has the potential to lead to extensive destruction of the interlobular bile ducts. A similar type of ductopenia observed after orthotopic liver transplantation, the vanishing bile duct syndrome, is a form of chronic liver allograft rejection in which bile duct injury and loss are thought to be mediated by a T-cell allogeneic response.Although an autoimmune mechanism may underlie the biliary duct injury in IAD, no specific evidence has been presented linking an immune-mediated attack and biliary ductal destruction in IAD.We analyzed the published data on IADand concluded that there are probably 2 variants of IAD, which may represent extremes of a disease spectrum. It would appear that the severity of the cholestasis, cirrhosis, and portal hypertension may be dependent on the degree of ductopenia. As evidenced by 2 recent series, patients with mild to moderate scarcity of intrahepatic biliary ducts, ie, less than 50% loss on a liver biopsy specimen, have a better prognosis and a more benign course than those having more extensive biliary duct destruction.Patients in this latter group with severe ductopenia frequently developed biliary cirrhosis and tend to ultimately require liver transplantation as definitive therapy. The clinical appearance of these 2 groups of patients seems to reflect different degrees of pathologic severity, which may require different management strategies. Interestingly, some of the more severe cases of ductopenia have often been described in males, in contrast to the patient described herein.Idiopathic adulthood ductopenia probably does not constitute a homogeneous entity. Its prognosis can be ominous at times. In patients with IAD who demonstrate progressive symptoms of liver failure, or intractable cholestatic symptoms such as refractory severe pruritus, orthotopic liver transplantation offers the only known cure, as shown in the patient described herein.In patients with nonprogressive disease, supportive care and monitoring may be all that is required.Clearly, further studies are needed to elucidate the cause of this disease.JLudwigIdiopathic adulthood ductopenia: an update.Mayo Clin Proc.1998;73:285-291.LLudwigRHWiesnerNFLaRussoIdiopathic adulthood ductopenia: a cause of chronic cholestatic liver disease and biliary cirrhosis.J Hepatol.1988;7:193-199.SSherlockThe syndrome of disappearing intrahepatic bile ducts.Lancet.1987;2:493-496.NVBergasaDWAllingTLTalbotEffects of naloxone infusions in patients with pruritus of cholestasis: a double-blind, randomized, controlled trial.Ann Intern Med.1995;123:161-167.AMorenoVCarrenoACanoCGonzalezIdiopathic biliary ductopenia in adults without symptoms of liver disease.N Engl J Med.1997;336:835-838.ESZafraniJMMetreauCDouvinIdiopathic biliary ductopenia in adults: a report of five cases.Gastroenterology.1990;99:1823-1828.JHaratakeAHorieNIshiiFOkunoFamilial intrahepatic cholestatic cirrhosis in young adults.Gastroenterology.1985;89:202-209.GBallardiniRMirakianFBRianchiAberrant expression of HLA-DR antigens on bile duct epithelium in primary biliary cirrhosis: relevance to pathogenesis.Lancet.1984;2:1009-1013.JPSloaneMJGFarthingRLPowlesHistopathological changes in the liver after allogenic bone marrow transplantation.J Clin Pathol.1980;33:344-350.FPAghaTTNostrantGDAbramsCytomegalovirus cholangitis in a homosexual man with acquired immune deficiency syndrome.Am J Gastroenterol.1986;81:1068-1072.VJDesmetDestructive intrahepatic bile duct diseases.Recenti Prog Med.1990;81:392-398.HJZymmermanDrug-induced liver disease.In: Schiff ER, Sorrell M, Maddrey WC, eds. Schiff's Diseases of the Liver. 8th ed. Philadelphia, Pa: Lippincott-Raven Publishers; 1999:973-1064.PJScheuerDrugs and toxins.In: Scheuer PJ, ed. Liver Biopsy Interpretation. London, England: Bailliere Tindall; 1988:99-112.DAlagilleAEstradaMHadchouelMGautierMOdievreJPDommerguesSyndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases.J Pediatr.1987;110:195-200.MBrugueraJLlachJRodesNonsyndromic paucity of intrahepatic bile ducts in infancy and idiopathic ductopenia in adulthood: the same syndrome?Hepatology.1992;15:830-834.MEsteveECondomVVillalbaFFernandez-BanaresFGonzalezCBadosaIntrahepatic bile duct hypoplasia: a report on two cases.J Clin Nutr Gastroenterol.1986;1:271-278.DAlagilleManagement of paucity of interlobular bile ducts.J Hepatol.1985;1:561-565.JTGalambosWSBrooks JrAtypical biliary cirrhosis or sclerosing cholangitis.J Clin Gastroenterol.1980;2:43-52.JLudwigRHWiesnerKPBattsJDPerkinsRAKromThe acute vanishing bile duct syndrome (acute irreversible rejection) after orthotopic liver transplantation.Hepatology.1987;7:476-483.CVPayaRHWiesnerPEHermansLack of association between cytomegalovirus infection, HLA matching and the vanishing bile duct syndrome after liver transplantation.Hepatology.1992;16:66-70.ELRCançadoSRSpinosaRMLMuszkatChronic cholestasis with ductopenia in adults [abstract].Hepatology.1992;16:521.HHartmannHJGroneIdiopathic ductopenia of adulthood: favorable effect of ursodeoxycholic acid therapy [in German].Z Gastroenterol.1993;31(suppl 2):131-133.JLudwigCBRosenKDLindorJHHelzbergKRWatsonChronic cholestasis in a young man.Hepatology.1994;20:1351-1355.KNakajimaMKomastsuTOnoA case of idiopathic adulthood ductopenia.Endoscopy.1994;26:332-333.CMullerWUlrichEPennerManifestation late in life of idiopathic adulthood ductopenia.Liver.1995;15:213-218.INakanoYFukudaYKoyamaIdiopathic adulthood ductopenia.J Gastroenterol Hepatol.1996;11:411-415.GFaaPVan EykenLDemeliaEVallebonaVCostaVJDesmetIdiopathic adulthood ductopenia presenting with chronic recurrent cholestasis: a case report.J Hepatol.1991;12:14-20.RJTuthillWDCareyELWinkelmanIdiopathic adulthood ductopenia [abstract].Lab Invest.1989;60:99A.Accepted for publication October 4, 1999.Reprints: Santiago J. Muñoz, MD, Albert Einstein Medical Center, 5401 Old York Rd, Klein Bldg, Suite 509, Philadelphia, PA 19141 (e-mail: munozs@aehn2.einstein.edu). http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Internal Medicine American Medical Association

Idiopathic Adulthood Ductopenia

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American Medical Association
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Copyright 2000 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
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Abstract

The clinical and pathological findings of idiopathic ductopenia were studied in a 30-year-old woman who initially manifested jaundice and pruritus. Serum biochemical tests of liver function indicated severe and progressive cholestasis. Viral hepatitis markers and circulating autoantibodies were absent. The patient had a normal cholangiogram and lacked evidence of inflammatory bowel disease. Histological examination of a liver specimen showed severe cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination. Transplantation resulted in prompt and complete resolution of the jaundice and pruritus. Two types of idiopathic adulthood ductopenia associated with different prognoses are recognized. Patients with type 1 idiopathic adulthood ductopenia are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biopsy specimens. Their clinical course ranges from spontaneous improvement to progression to biliary cirrhosis. In contrast, patients with type 2 idiopathic adulthood ductopenia generally manifest initial symptoms of decompensated biliary cirrhosis, have extensive destruction of the intrahepatic bile ducts on liver biopsy, and frequently require orthotopic liver transplantation.Idiopathic adulthood ductopenia (IAD) is a disease of unknown cause that affects predominantly young adults and is characterized by clinical and biochemical evidence of cholestasis.The hallmark of IAD is the loss of interlobular and septal bile ducts observed on liver biopsy specimens.Since the first description in 1988,several cases of IAD have been reported, yet the pathogenesis remains unknown.In the absence of definite and specific diagnostic criteria, IAD remains a diagnosis of exclusion, to be considered in patients with cholestatic liver disease of obscure cause. Herein we describe a patient with IAD and review the literature to date on this uncommon hepatobiliary disorder.REPORT OF A CASEA 30-year-old Filipino woman who had lived in the United States since 1995 was admitted to our institution in September 1997 with gradually progressive fatigue, jaundice, and severe, generalized pruritus of 4 months' duration. Her medical history consisted only of a cholecystectomy for cholelithiasis and chronic cholecystitis 3 years earlier. She had not had neonatal or childhood jaundice, viral hepatitis, other previous liver disease, alcohol or other drug abuse, or transfusions of blood products. She had no relevant family or social history. The patient denied ingestion during the preceding 12 months of chlorpromazine, prochlorperazine, tolbutamide, or organic arsenicals. On admission, physical examination showed marked scleral icterus and diffuse skin hyperpigmentation. No stigmas of chronic liver disease or xanthelasmas were apparent. Her abdomen was soft and without tenderness. There was no evidence of hepatosplenomegaly, ascites, or prominent collateral venous circulation.Laboratory studies showed normal white and red blood cell counts and a prolonged prothrombin time (21 seconds; reference range, 11-13 seconds). Serum biochemical tests of liver function showed a predominantly cholestatic pattern: total serum bilirubin level, 257 µmol/L (15 mg/dL) (reference range, 0-21 µmol/L [0-1.2 mg/dL]); direct bilirubin level, 136.8 µmol/L (8 mg/dL) (reference range, 0-6.8 µmol/L [0-0.4 mg/dL]); γ-glutamyltransferase level, 250 U/L (reference range, 8-78 U/L); alkaline phosphatase level, 300 U/L (reference range, 34-124 U/L); and elevated aminotransferase levels: aspartate aminotransferase level, 160 U/L (reference range, 8-54 U/L) and alanine aminotransferase level, 210 U/L (reference range, 8-52 U/L). Hepatitis A antibody, hepatitis B surface antigen and antibody, hepatitis B core antibody, hepatitis C antibody, amylase, lipase, α1-antitrypsin, iron, ferritin, and ceruloplasmin levels were all absent or within normal limits. Tests for antinuclear, anti-DNA, antimitochondrial, anti–liver-kidney microsomal type 1, and anti–smooth muscle autoantibodies and rheumatoid factor were all negative as well.Ultrasound of the abdomen demonstrated mild hepatomegaly, without biliary ductal dilatation or parenchymal abnormalities. Endoscopic retrograde cholangiopancreatography showed a normal biliary tract and pancreatic ductal anatomy. Esophagogastroduodenoscopy and colonoscopy were normal. A percutaneous liver biopsy specimen showed a striking absence of interlobular bile ducts, marked cholestasis, portal inflammation, and fibrosis (Figure 1and Figure 2). Biliary cirrhosis secondary to IAD was diagnosed.Figure 1.Pathological analysis of the explanted liver. Macroscopically, the liver exhibited an intense dark-green color, consistent with severe chronic cholestasis. At lower power, absence of interlobular bile ducts in the portal area and severe cholestasis, particularly in zone 1 of the hepatic lobule (periportal), are observed (hematoxylin-eosin, original magnification ×200).Figure 2.Higher-power view of a portal tract confirming the absence of bile ducts and the presence of a mild chronic inflammatory infiltrate that includes lymphocytes, neutrophils, and plasma cells (hematoxylin-eosin, original magnification ×400).The patient's jaundice worsened, with a bilirubin level greater than 342 µmol/L (20 mg/dL). Her pruritus became severe and refractory to the use of cholestyramine resin, ursodiol, rifampin, and even continuous infusions of intravenous naloxone hydrochloride.She developed disabling fatigue. She underwent successful orthotopic liver transplantation, which was followed by prompt disappearance of the pruritus, fatigue, and jaundice. Her postoperative course was uncomplicated, and at follow-up 12 months after transplantation, she was doing well and was free of symptoms, with normal results of liver function studies and no evidence of allograft dysfunction.COMMENTThe case described herein demonstrates the hallmark characteristics of the IAD syndrome, first described by Ludwig et al in 1988.Idiopathic adulthood ductopenia is a chronic cholestatic liver disease of unknown cause characterized by the loss of interlobular and septal bile ducts.Numerous known causes have been implicated in the pathogenesis of intrahepatic biliary destruction. These include developmental biliary atresia (secondary to infection or α1-antitrypsin deficiency),immunologic causes resulting from aberrant expression of HLA class II histocompatibility antigens expressed on the surface of biliary epithelial cells (such as might be the case in primary sclerosing cholangitis, graft vs host disease, sarcoidosis, and primary biliary cirrhosis),infection (cholangitis),ischemia, and chemical injuries (such as injection of scolicidal solution or formaldehyde).Furthermore, a variety of therapeutic drugs have occasionally been associated with bile duct destruction and loss, including chlorpromazine, prochlorperazine, organic arsenicals, and tolbutamide.If these causes are systematically excluded, IAD should be considered as the possible diagnosis. This entity occurs in young to middle-aged adults, without history of infantile cholangiopathy, who manifest cholestatic liver disease. The following criteria are necessary: absence of interlobular bile ducts in at least 50% of small portal tracts on an adequate-sized liver biopsy specimen, normal endoscopic retrograde cholangiopancreatography (thus excluding disorders of medium-size and large biliary ducts, such as primary sclerosing cholangitis), normal colonoscopy (to exclude inflammatory bowel disease–related cholangiopathy), absence of antimitochondrial antibodies (excluding primary biliary cirrhosis), and absence of granulomatous cholangitis, histiocytosis X, neutrophilic suppurative cholangitis, lymphoma, or neoplasm in liver biopsy specimens.Although a distinct cause of IAD has not been determined, several hypotheses have been raised. Some investigators have suggested that IAD may be related to the late expression of a nonsyndromic form of Alagille syndrome (infantile scarcity of interlobular bile ducts), an uncommon disease of childhood.This syndrome is characterized by atresia of interlobular bile ducts and is associated with a combination of the following: chronic cholestasis, triangular face, prominent forehead and hypertelorism, posterior embryotoxon, vertebral arch defects, and peripheral pulmonary hypoplasia.However, IAD shares few characteristics with the nonsyndromic form of Alagille syndromeand lacks the relatively poor prognosis. Half of the patients with the nonsyndromic form of Alagille syndrome develop cirrhosis with portal hypertension and die of liver failure within the first years of life.It is also possible that some patients with IAD have a form of viral cholangitis; however, no specific viral agent has been isolated in affected individuals. Although many patients with IAD have had cholecystectomy, a role for previous bacterial ascending cholangitis has not been proved. The clinical appearance of patients with IAD bears similarities to that of patients with primary sclerosing cholangitis. In fact, pericholangitis is frequently observed surrounding the remaining bile ducts in liver biopsy specimens from patients with IAD. Thus, it is conceivable that IAD could be considered a form of isolated, atypical, or limited variant of small-duct primary sclerosing cholangitisthat nevertheless has the potential to lead to extensive destruction of the interlobular bile ducts. A similar type of ductopenia observed after orthotopic liver transplantation, the vanishing bile duct syndrome, is a form of chronic liver allograft rejection in which bile duct injury and loss are thought to be mediated by a T-cell allogeneic response.Although an autoimmune mechanism may underlie the biliary duct injury in IAD, no specific evidence has been presented linking an immune-mediated attack and biliary ductal destruction in IAD.We analyzed the published data on IADand concluded that there are probably 2 variants of IAD, which may represent extremes of a disease spectrum. It would appear that the severity of the cholestasis, cirrhosis, and portal hypertension may be dependent on the degree of ductopenia. As evidenced by 2 recent series, patients with mild to moderate scarcity of intrahepatic biliary ducts, ie, less than 50% loss on a liver biopsy specimen, have a better prognosis and a more benign course than those having more extensive biliary duct destruction.Patients in this latter group with severe ductopenia frequently developed biliary cirrhosis and tend to ultimately require liver transplantation as definitive therapy. The clinical appearance of these 2 groups of patients seems to reflect different degrees of pathologic severity, which may require different management strategies. Interestingly, some of the more severe cases of ductopenia have often been described in males, in contrast to the patient described herein.Idiopathic adulthood ductopenia probably does not constitute a homogeneous entity. Its prognosis can be ominous at times. In patients with IAD who demonstrate progressive symptoms of liver failure, or intractable cholestatic symptoms such as refractory severe pruritus, orthotopic liver transplantation offers the only known cure, as shown in the patient described herein.In patients with nonprogressive disease, supportive care and monitoring may be all that is required.Clearly, further studies are needed to elucidate the cause of this disease.JLudwigIdiopathic adulthood ductopenia: an update.Mayo Clin Proc.1998;73:285-291.LLudwigRHWiesnerNFLaRussoIdiopathic adulthood ductopenia: a cause of chronic cholestatic liver disease and biliary cirrhosis.J Hepatol.1988;7:193-199.SSherlockThe syndrome of disappearing intrahepatic bile ducts.Lancet.1987;2:493-496.NVBergasaDWAllingTLTalbotEffects of naloxone infusions in patients with pruritus of cholestasis: a double-blind, randomized, controlled trial.Ann Intern Med.1995;123:161-167.AMorenoVCarrenoACanoCGonzalezIdiopathic biliary ductopenia in adults without symptoms of liver disease.N Engl J Med.1997;336:835-838.ESZafraniJMMetreauCDouvinIdiopathic biliary ductopenia in adults: a report of five cases.Gastroenterology.1990;99:1823-1828.JHaratakeAHorieNIshiiFOkunoFamilial intrahepatic cholestatic cirrhosis in young adults.Gastroenterology.1985;89:202-209.GBallardiniRMirakianFBRianchiAberrant expression of HLA-DR antigens on bile duct epithelium in primary biliary cirrhosis: relevance to pathogenesis.Lancet.1984;2:1009-1013.JPSloaneMJGFarthingRLPowlesHistopathological changes in the liver after allogenic bone marrow transplantation.J Clin Pathol.1980;33:344-350.FPAghaTTNostrantGDAbramsCytomegalovirus cholangitis in a homosexual man with acquired immune deficiency syndrome.Am J Gastroenterol.1986;81:1068-1072.VJDesmetDestructive intrahepatic bile duct diseases.Recenti Prog Med.1990;81:392-398.HJZymmermanDrug-induced liver disease.In: Schiff ER, Sorrell M, Maddrey WC, eds. Schiff's Diseases of the Liver. 8th ed. Philadelphia, Pa: Lippincott-Raven Publishers; 1999:973-1064.PJScheuerDrugs and toxins.In: Scheuer PJ, ed. Liver Biopsy Interpretation. London, England: Bailliere Tindall; 1988:99-112.DAlagilleAEstradaMHadchouelMGautierMOdievreJPDommerguesSyndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases.J Pediatr.1987;110:195-200.MBrugueraJLlachJRodesNonsyndromic paucity of intrahepatic bile ducts in infancy and idiopathic ductopenia in adulthood: the same syndrome?Hepatology.1992;15:830-834.MEsteveECondomVVillalbaFFernandez-BanaresFGonzalezCBadosaIntrahepatic bile duct hypoplasia: a report on two cases.J Clin Nutr Gastroenterol.1986;1:271-278.DAlagilleManagement of paucity of interlobular bile ducts.J Hepatol.1985;1:561-565.JTGalambosWSBrooks JrAtypical biliary cirrhosis or sclerosing cholangitis.J Clin Gastroenterol.1980;2:43-52.JLudwigRHWiesnerKPBattsJDPerkinsRAKromThe acute vanishing bile duct syndrome (acute irreversible rejection) after orthotopic liver transplantation.Hepatology.1987;7:476-483.CVPayaRHWiesnerPEHermansLack of association between cytomegalovirus infection, HLA matching and the vanishing bile duct syndrome after liver transplantation.Hepatology.1992;16:66-70.ELRCançadoSRSpinosaRMLMuszkatChronic cholestasis with ductopenia in adults [abstract].Hepatology.1992;16:521.HHartmannHJGroneIdiopathic ductopenia of adulthood: favorable effect of ursodeoxycholic acid therapy [in German].Z Gastroenterol.1993;31(suppl 2):131-133.JLudwigCBRosenKDLindorJHHelzbergKRWatsonChronic cholestasis in a young man.Hepatology.1994;20:1351-1355.KNakajimaMKomastsuTOnoA case of idiopathic adulthood ductopenia.Endoscopy.1994;26:332-333.CMullerWUlrichEPennerManifestation late in life of idiopathic adulthood ductopenia.Liver.1995;15:213-218.INakanoYFukudaYKoyamaIdiopathic adulthood ductopenia.J Gastroenterol Hepatol.1996;11:411-415.GFaaPVan EykenLDemeliaEVallebonaVCostaVJDesmetIdiopathic adulthood ductopenia presenting with chronic recurrent cholestasis: a case report.J Hepatol.1991;12:14-20.RJTuthillWDCareyELWinkelmanIdiopathic adulthood ductopenia [abstract].Lab Invest.1989;60:99A.Accepted for publication October 4, 1999.Reprints: Santiago J. Muñoz, MD, Albert Einstein Medical Center, 5401 Old York Rd, Klein Bldg, Suite 509, Philadelphia, PA 19141 (e-mail: munozs@aehn2.einstein.edu).

Journal

JAMA Internal Medicine – American Medical Association

Published: Apr 10, 2000

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