Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia

Background:Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by progressive inflammation and fibrosis of the biliary tract, evolving to cirrhosis. It is commonly associated with inflammatory bowel disease (IBD). Aim: To communicate the clinical characteristics of p...

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Autores principales:	Orellana N,Ivonne, Valera M,José Miguel, Nei L,Chun, Poniachik T,Jaime, Berger F,Zoltán, Latorre M,Ricardo, Smok S,Gladys, Brahm B,Javier
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2005
Materias:	Cholangitis sclerosing Cholestasis, intraheaptic Hepatitis, autoimmune Ursodeoxycholic acid
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005000700004
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spelling	oai:scielo:S0034-988720050007000042014-08-12Colangitis esclerosante primaria: revisión de 12 años en dos centros de referenciaOrellana N,IvonneValera M,José MiguelNei L,ChunPoniachik T,JaimeBerger F,ZoltánLatorre M,RicardoSmok S,GladysBrahm B,Javier Cholangitis sclerosing Cholestasis, intraheaptic Hepatitis, autoimmune Ursodeoxycholic acid Background:Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by progressive inflammation and fibrosis of the biliary tract, evolving to cirrhosis. It is commonly associated with inflammatory bowel disease (IBD). Aim: To communicate the clinical characteristics of patient with PSC seen in two reference centers. Patients and methods: Review of medical records of patients with PSC confirmed by liver biopsies. The clinical picture, laboratory abnormalities, imaging studies and treatment were recorded. Results: Thirty three patients (aged 16 to 73 years, 64% female) were identified. They corresponded to 1.7% of liver biopsies done between 1991-2003. Clinical presentation was a cholestatic picture in 40%, right upper abdominal pain in 36%, a dysenteric syndrome in 9% and varied symptoms in 15%. Laboratory tests showed cholestasis in 94% and positive anti ANCA, SMA, ANA and AMA antibodies in 28, 18, 15 and 9% of cases, respectively. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography were diagnostic in 43 and 58% of patients, respectively. There was an association with ulcerative colitis in 12% of cases. Liver biopsies showed grade I PSC in 76% and grade II-III in 6% of patients. It also showed a concomitant chronic hepatitis and primary biliary cirrhosis in 12 and 6% of cases, respectively. Treatment consisted on ursodeoxycholic acid (UDCA) in 45%, UDCA plus 5-aminosalicylic acid derivatives in 12% and UDCA plus immunosuppresors in 12% of patients. Two patients had to be transplanted. Conclusions: PSC is an uncommon cause of chronic liver disease. It is suspected in cholestatic patients and confirmed with a liver biopsy. It can be associated with other autoimmune hepatic and extrahepatic diseasesinfo:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.133 n.7 20052005-07-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005000700004es10.4067/S0034-98872005000700004
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Cholangitis sclerosing Cholestasis, intraheaptic Hepatitis, autoimmune Ursodeoxycholic acid
spellingShingle	Cholangitis sclerosing Cholestasis, intraheaptic Hepatitis, autoimmune Ursodeoxycholic acid Orellana N,Ivonne Valera M,José Miguel Nei L,Chun Poniachik T,Jaime Berger F,Zoltán Latorre M,Ricardo Smok S,Gladys Brahm B,Javier Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia
description	Background:Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by progressive inflammation and fibrosis of the biliary tract, evolving to cirrhosis. It is commonly associated with inflammatory bowel disease (IBD). Aim: To communicate the clinical characteristics of patient with PSC seen in two reference centers. Patients and methods: Review of medical records of patients with PSC confirmed by liver biopsies. The clinical picture, laboratory abnormalities, imaging studies and treatment were recorded. Results: Thirty three patients (aged 16 to 73 years, 64% female) were identified. They corresponded to 1.7% of liver biopsies done between 1991-2003. Clinical presentation was a cholestatic picture in 40%, right upper abdominal pain in 36%, a dysenteric syndrome in 9% and varied symptoms in 15%. Laboratory tests showed cholestasis in 94% and positive anti ANCA, SMA, ANA and AMA antibodies in 28, 18, 15 and 9% of cases, respectively. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography were diagnostic in 43 and 58% of patients, respectively. There was an association with ulcerative colitis in 12% of cases. Liver biopsies showed grade I PSC in 76% and grade II-III in 6% of patients. It also showed a concomitant chronic hepatitis and primary biliary cirrhosis in 12 and 6% of cases, respectively. Treatment consisted on ursodeoxycholic acid (UDCA) in 45%, UDCA plus 5-aminosalicylic acid derivatives in 12% and UDCA plus immunosuppresors in 12% of patients. Two patients had to be transplanted. Conclusions: PSC is an uncommon cause of chronic liver disease. It is suspected in cholestatic patients and confirmed with a liver biopsy. It can be associated with other autoimmune hepatic and extrahepatic diseases
author	Orellana N,Ivonne Valera M,José Miguel Nei L,Chun Poniachik T,Jaime Berger F,Zoltán Latorre M,Ricardo Smok S,Gladys Brahm B,Javier
author_facet	Orellana N,Ivonne Valera M,José Miguel Nei L,Chun Poniachik T,Jaime Berger F,Zoltán Latorre M,Ricardo Smok S,Gladys Brahm B,Javier
author_sort	Orellana N,Ivonne
title	Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia
title_short	Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia
title_full	Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia
title_fullStr	Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia
title_full_unstemmed	Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia
title_sort	colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia
publisher	Sociedad Médica de Santiago
publishDate	2005
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005000700004
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Colangitis esclerosante primaria: revisión de 12 años en dos centros de referencia

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