Research ExplorerPublicationsCharacteristics and long-term prognosis of the autoimmune he...

Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome

Standard

Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. / Lüth, Stefan; Kanzler, Stephan; Frenzel, Christian; Kasper, Hans; Dienes, Hans; Schramm, Christoph; Galle, Peter; Herkel, Johannes; Lohse, Ansgar W.

In: J CLIN GASTROENTEROL, Vol. 43, No. 1, 01.2009, p. 75-80.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Lüth, S, Kanzler, S, Frenzel, C, Kasper, H, Dienes, H, Schramm, C, Galle, P, Herkel, J & Lohse, AW 2009, 'Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome', J CLIN GASTROENTEROL, vol. 43, no. 1, pp. 75-80. https://doi.org/10.1097/MCG.0b013e318157c614

APA

Lüth, S., Kanzler, S., Frenzel, C., Kasper, H., Dienes, H., Schramm, C., Galle, P., Herkel, J., & Lohse, A. W. (2009). Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. J CLIN GASTROENTEROL, 43(1), 75-80. https://doi.org/10.1097/MCG.0b013e318157c614

Vancouver

Lüth S, Kanzler S, Frenzel C, Kasper H, Dienes H, Schramm C et al. Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. J CLIN GASTROENTEROL. 2009 Jan;43(1):75-80. https://doi.org/10.1097/MCG.0b013e318157c614

Bibtex

@article{16861c0a8b8b4ad7844af07b4640037f,
title = "Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome",
abstract = "GOALS/BACKGROUND: Diagnosis, treatment, and prognosis of the overlap syndrome of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are controversial. Our aim was to assess the clinical characteristics and long-term prognosis of the AIH/PSC overlap syndrome. STUDY: We reviewed the data of 16 patients seen in our center who fulfilled the diagnostic criteria of both diseases at some stage of their medical history. RESULTS: All patients had initially presented with laboratory markers of both, cholestasis and definite AIH. Histologic reexamination of initial biopsies, available from 11 of 16 patients, revealed features of both AIH and PSC in all biopsies. Cholangiography was performed at initial presentation in 9 of 16 patients and appeared normal in 6 of 9 patients. During follow-up cholangiography, an additional 11 patients developed pathologic characteristics of PSC. The age and sex distribution was typical for PSC. Immunosuppressive therapy improved biochemical markers; however, fibrosis was observed to progress in all patients during a median observation period of 12 years. Three patients initially presented with cirrhosis, 12 of 16 patients developed cirrhosis at the end of the observation period, and 3 developed complications of cirrhosis. CONCLUSIONS: Overlap of AIH and PSC was detected most reliably on grounds of serologic markers and histology; early bile duct changes were often missed by endoscopic retrograde cholangiography. Immunosuppression combined with ursodeoxycholic acid seems to be beneficial, but cannot prevent long-term progression toward cirrhosis in the majority of patients.",
keywords = "Adolescent, Adult, Aged, Cholangiography, Cholangitis, Sclerosing, Disease Progression, Drug Therapy, Combination, Female, Fibrosis, Follow-Up Studies, Hepatitis, Autoimmune, Humans, Immunosuppressive Agents, Liver Cirrhosis, Male, Middle Aged, Prognosis, Retrospective Studies, Syndrome, Ursodeoxycholic Acid, Young Adult, Journal Article, Research Support, Non-U.S. Gov't",
author = "Stefan L{\"u}th and Stephan Kanzler and Christian Frenzel and Hans Kasper and Hans Dienes and Christoph Schramm and Peter Galle and Johannes Herkel and Lohse, {Ansgar W.}",
year = "2009",
month = jan,
doi = "10.1097/MCG.0b013e318157c614",
language = "English",
volume = "43",
pages = "75--80",
journal = "J CLIN GASTROENTEROL",
issn = "0192-0790",
publisher = "Lippincott Williams and Wilkins",
number = "1",

}

RIS

TY - JOUR

T1 - Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome

AU - Lüth, Stefan

AU - Kanzler, Stephan

AU - Frenzel, Christian

AU - Kasper, Hans

AU - Dienes, Hans

AU - Schramm, Christoph

AU - Galle, Peter

AU - Herkel, Johannes

AU - Lohse, Ansgar W.

PY - 2009/1

Y1 - 2009/1

N2 - GOALS/BACKGROUND: Diagnosis, treatment, and prognosis of the overlap syndrome of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are controversial. Our aim was to assess the clinical characteristics and long-term prognosis of the AIH/PSC overlap syndrome. STUDY: We reviewed the data of 16 patients seen in our center who fulfilled the diagnostic criteria of both diseases at some stage of their medical history. RESULTS: All patients had initially presented with laboratory markers of both, cholestasis and definite AIH. Histologic reexamination of initial biopsies, available from 11 of 16 patients, revealed features of both AIH and PSC in all biopsies. Cholangiography was performed at initial presentation in 9 of 16 patients and appeared normal in 6 of 9 patients. During follow-up cholangiography, an additional 11 patients developed pathologic characteristics of PSC. The age and sex distribution was typical for PSC. Immunosuppressive therapy improved biochemical markers; however, fibrosis was observed to progress in all patients during a median observation period of 12 years. Three patients initially presented with cirrhosis, 12 of 16 patients developed cirrhosis at the end of the observation period, and 3 developed complications of cirrhosis. CONCLUSIONS: Overlap of AIH and PSC was detected most reliably on grounds of serologic markers and histology; early bile duct changes were often missed by endoscopic retrograde cholangiography. Immunosuppression combined with ursodeoxycholic acid seems to be beneficial, but cannot prevent long-term progression toward cirrhosis in the majority of patients.

AB - GOALS/BACKGROUND: Diagnosis, treatment, and prognosis of the overlap syndrome of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are controversial. Our aim was to assess the clinical characteristics and long-term prognosis of the AIH/PSC overlap syndrome. STUDY: We reviewed the data of 16 patients seen in our center who fulfilled the diagnostic criteria of both diseases at some stage of their medical history. RESULTS: All patients had initially presented with laboratory markers of both, cholestasis and definite AIH. Histologic reexamination of initial biopsies, available from 11 of 16 patients, revealed features of both AIH and PSC in all biopsies. Cholangiography was performed at initial presentation in 9 of 16 patients and appeared normal in 6 of 9 patients. During follow-up cholangiography, an additional 11 patients developed pathologic characteristics of PSC. The age and sex distribution was typical for PSC. Immunosuppressive therapy improved biochemical markers; however, fibrosis was observed to progress in all patients during a median observation period of 12 years. Three patients initially presented with cirrhosis, 12 of 16 patients developed cirrhosis at the end of the observation period, and 3 developed complications of cirrhosis. CONCLUSIONS: Overlap of AIH and PSC was detected most reliably on grounds of serologic markers and histology; early bile duct changes were often missed by endoscopic retrograde cholangiography. Immunosuppression combined with ursodeoxycholic acid seems to be beneficial, but cannot prevent long-term progression toward cirrhosis in the majority of patients.

KW - Adolescent

KW - Adult

KW - Aged

KW - Cholangiography

KW - Cholangitis, Sclerosing

KW - Disease Progression

KW - Drug Therapy, Combination

KW - Female

KW - Fibrosis

KW - Follow-Up Studies

KW - Hepatitis, Autoimmune

KW - Humans

KW - Immunosuppressive Agents

KW - Liver Cirrhosis

KW - Male

KW - Middle Aged

KW - Prognosis

KW - Retrospective Studies

KW - Syndrome

KW - Ursodeoxycholic Acid

KW - Young Adult

KW - Journal Article

KW - Research Support, Non-U.S. Gov't

U2 - 10.1097/MCG.0b013e318157c614

DO - 10.1097/MCG.0b013e318157c614

M3 - SCORING: Journal article

C2 - 18769363

VL - 43

SP - 75

EP - 80

JO - J CLIN GASTROENTEROL

JF - J CLIN GASTROENTEROL

SN - 0192-0790

IS - 1

ER -