Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome

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Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome

Efe, C. (författare): Department of Gastroenterology, Gazi Yaşargil Education and Research Hospital, Diyarbakir, Turkey,Gazi Yasargil Educ & Res Hosp, Dept Gastroenterol, Diyarbakir, Turkey.

Torgutalp, M. (författare): Department of Rheumatology, Ankara University Hospital, Ankara, Turkey,Ankara Univ Hosp, Dept Rheumatol, Ankara, Turkey.

Henriksson, Ida, 1980- (författare): Örebro universitet,Institutionen för medicinska vetenskaper,Department of Gastroenterology,Örebro Univ, Fac Med & Hlth, Dept Gastroenterol, Örebro, Sweden.

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Alalkim, F. (författare): Division of Gastroenterology, University of British Columbia and Vancouver General Hospital, Vancouver BC, Canada,Univ British Columbia, Div Gastroenterol, Vancouver, BC, Canada.;Vancouver Gen Hosp, Vancouver, BC, Canada.

Lytvyak, E. (författare): University of Alberta Division of Gastroenterology and Liver Unit, Edmonton AB, Canada,Univ Alberta, Div Gastroenterol, Edmonton, AB, Canada.;Univ Alberta, Liver Unit, Edmonton, AB, Canada.

Trivedi, H. (författare): Division of GI and Hepatology, Beth Israel Medical Center, Harvard Medical School, Boston MA, United States,Harvard Med Sch, Beth Israel Med Ctr, Div GI & Hepatol, Boston, MA 02115 USA.

Eren, F. (författare): Department of Gastroenterology, Medical Faculty, Uludag University, Bursa, Turkey,Uludag Univ, Med Fac, Dept Gastroenterol, Bursa, Turkey.

Fischer, J. (författare): Division of Gastroenterology, Clinic and Polyclinic for Oncology, Hepatology, Infectious Diseases and Pneumology, University Clinic Leipzig, Leipzig, Germany,Univ Clin Leipzig, Div Gastroenterol, Clin & Polyclin Oncol Hepatol Infect Dis & Pneumo, Leipzig, Germany.

Chayanupatkul, M. (författare): Division of Liver Diseases, The Mount Sinai Medical Center, New York NY, United States; Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand,Chulalongkorn Univ, Fac Med, Bangkok, Thailand.

Coppo, C. (författare): Center for the Study and Treatment of Autoimmune Diseases of the Liver and Biliary System, University of Bologna, Bologna, Italy,Univ Bologna, Ctr Study & Treatment Autoimmune Dis Liver & Bina, Bologna, Italy.

Purnak, T. (författare): Department of Gastroenterology, Hacettepe University, Ankara, Turkey,Hacettepe Univ, Dept Gastroenterol, Ankara, Turkey.

Muratori, L. (författare): Center for the Study and Treatment of Autoimmune Diseases of the Liver and Biliary System, University of Bologna, Bologna, Italy,Univ Bologna, Ctr Study & Treatment Autoimmune Dis Liver & Bina, Bologna, Italy.

Werner, Mårten (författare): Umeå universitet,Avdelningen för medicin,Umeå Univ, Dept Publ Hlth & Clin Med, Umeå, Sweden.

Muratori, P. (författare): Center for the Study and Treatment of Autoimmune Diseases of the Liver and Biliary System, University of Bologna, Bologna, Italy,Univ Bologna, Ctr Study & Treatment Autoimmune Dis Liver & Bina, Bologna, Italy.

Rorsman, Fredrik, Docent, 1960- (författare): Uppsala universitet,Gastroenterologi/hepatologi

Onnerhag, K. (författare): Department of Gastroenterology and Hepatology, Skåne University Hospital, Malmö, Sweden,Skane Univ Hosp, Dept Gastroenterol & Hepatol, Malmö, Sweden.

Nilsson, E. (författare): Department of Clinical Sciences, Gastroenterology Division, Skåne University Hospital, Lund, Sweden,Skane Univ Hosp, Dept Clin Sci, Gastroenterol Div, Lund, Sweden.

Heurgue-Berlot, A. (författare): Department of Hepato-Gastroenterology, CHU Reims, Reims, France,CHU Reims, Dept Hepatogastroenterol, Reims, France.

Demir, N. (författare): Department of Gastroenterology, Gazi Yaşargil Education and Research Hospital, Diyarbakir, Turkey,Gazi Yasargil Educ & Res Hosp, Dept Gastroenterol, Diyarbakir, Turkey.

Semela, D. (författare): Division of Gastroenterology and Hepatology, Kantonsspital St. Gallen, St. Gallen, Switzerland,Kantonsspital St Gallen, Div Gastroenterol & Hepatol, St Gallen, Switzerland.

Kiyici, M. (författare): Department of Gastroenterology, Medical Faculty, Uludag University, Bursa, Turkey,Uludag Univ, Med Fac, Dept Gastroenterol, Bursa, Turkey.

Schiano, T. D. (författare): Division of Liver Diseases, The Mount Sinai Medical Center, New York NY, United States,Mt Sinai Med Ctr, Div Liver Dis, New York, NY 10029 USA.

Montano-Loza, A. J. (författare): University of Alberta Division of Gastroenterology and Liver Unit, Edmonton AB, Canada,Univ Alberta, Div Gastroenterol, Edmonton, AB, Canada.;Univ Alberta, Liver Unit, Edmonton, AB, Canada.

Berg, T. (författare): Division of Gastroenterology, Clinic and Polyclinic for Oncology, Hepatology, Infectious Diseases and Pneumology, University Clinic Leipzig, Leipzig, Germany,Univ Clin Leipzig, Div Gastroenterol, Clin & Polyclin Oncol Hepatol Infect Dis & Pneumo, Leipzig, Germany.

Ozaslan, E. (författare): Department of Gastroenterology, Ankara City Hospital, Ankara, Turkey,Ankara City Hosp, Dept Gastroenterol, Ankara, Turkey.

Yoshida, E. M. (författare): Division of Gastroenterology, University of British Columbia and Vancouver General Hospital, Vancouver BC, Canada,Univ British Columbia, Div Gastroenterol, Vancouver, BC, Canada.;Vancouver Gen Hosp, Vancouver, BC, Canada.

Bonder, A. (författare): Division of GI and Hepatology, Beth Israel Medical Center, Harvard Medical School, Boston MA, United States,Harvard Med Sch, Beth Israel Med Ctr, Div GI & Hepatol, Boston, MA 02115 USA.

Marschall, Hanns-Ulrich, 1954 (författare): Gothenburg University,Göteborgs universitet,Institutionen för medicin, avdelningen för molekylär och klinisk medicin,Institute of Medicine, Department of Molecular and Clinical Medicine,Department of Molecular and Clinical Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden,Univ Gothenburg, Sahlgrenska Acad, Dept Mol & Clin Med, Gothenburg, Sweden.

Beretta-Piccoli, B. T. (författare): Epatocentro Ticino, Lugano, Switzerland,Epatoctr Ticino, Lugano, Switzerland.

Wahlin, S. (författare): Karolinska Institutet,Karolinska Inst, Ctr Digest Dis, Hepatol Div, Stockholm, Sweden.;Karolinska Univ Hosp, Stockholm, Sweden.

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Department of Gastroenterology, Gazi Yaşargil Education and Research Hospital, Diyarbakir, Turkey Gazi Yasargil Educ & Res Hosp, Dept Gastroenterol, Diyarbakir, Turkey (creator_code:org_t)

2020-08-23
2021
Engelska.
Ingår i: Journal of Gastroenterology and Hepatology. - : Wiley. - 0815-9319 .- 1440-1746. ; 36:4, s. 936-942

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Tidskriftsartikel (refereegranskat)

Abstract Ämnesord

Stäng

Background and Aim The prevalence and clinical significance of extrahepatic autoimmune diseases (EHAIDs) have not been evaluated in a large cohort of primary biliary cholangitis (PBC). Methods The medical records of 1554 patients with PBC from 20 international centers were retrospectively reviewed. Development of decompensated cirrhosis (ascites, variceal bleeding, and/or hepatic encephalopathy) and hepatocellular carcinoma were considered clinical endpoints. Results A total of 35 different EHAIDs were diagnosed in 440 (28.3%) patients with PBC. Patients with EHAIDs were more often female (92.5%vs86.1%,P < 0.001) and seropositive for anti-mitochondrial antibodies (88%vs84%,P = 0.05) and antinuclear antibodies and/or smooth muscle antibodies (53.8%vs43.6%,P = 0.005). At presentation, patients with EHAIDs had significantly lower levels of alkaline phosphatase (1.76vs1.98 x upper limit of normal [ULN],P = 0.006), aspartate aminotransferase (1.29vs1.50 x ULN,P < 0.001), and total bilirubin (0.53vs0.58 x ULN,P = 0.002). Patients with EHAIDs and without EHAIDs had similar rates of GLOBE high-risk status (12.3%vs16.1%,P = 0.07) and Paris II response (71.4%vs69.4%,P = 0.59). Overall, event-free survival was not different in patients with and without EHAIDs (90.8%vs90.7%,P = 0.53, log rank). Coexistence of each autoimmune thyroid diseases (10.6%), Sjogren disease (8.3%), systemic sclerosis (2.9%), rheumatoid arthritis (2.7%), systemic lupus erythematosus (1.7%), celiac disease (1.7%), psoriasis (1.5%), and inflammatory bowel diseases (1.3%) did not influence the outcome. Conclusions Our study confirms that EHAIDs are frequently diagnosed in patients with PBC. The presence of EHAIDs may influence the clinical phenotype of PBC at presentation but has no impact on PBC outcome.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Gastroenterologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Gastroenterology and Hepatology (hsv//eng)

Nyckelord

Ankylosing spondylitis
Anti-phospholipid syndrome
Autoimmune hemolytic
anemia
Idiopathic thrombocytopenic purpura
IgA nephropathy
Multiple
sclerosis
Polyarteritis nodosa
Polymyositis
Sarcoidosis
Temporal
arteritis
biochemical response
ursodeoxycholic acid
risk-factors
cirrhosis
prognosis
sarcoidosis
management
pbc
Gastroenterology & Hepatology
Ankylosing spondylitis

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