Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome
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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, Jahrgang 43, Nr. 1, 01.2009, S. 75-80.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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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 -