2022-12-192022-12-192020-08-13Efe, C. vd. (2020). "Extrahepatic autoimmune diseases in primary biliary cholangitis: Prevalence and significance for clinical presentation and disease outcome". Journal of Gastroenterology and Hepatology, 36(4), 936-942.0815-93191440-1746https://doi.org/10.1111/jgh.15214https://onlinelibrary.wiley.com/doi/10.1111/jgh.15214http://hdl.handle.net/11452/29966Çalışmada 25 yazar bulunmaktadır. Bu yazarlardan sadece Bursa Uludağ Üniversitesi mensuplarının girişleri yapılmıştır.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.eninfo:eu-repo/semantics/closedAccessAnkylosing spondylitisAnti-phospholipid syndromeAutoimmune hemolytic anemiaIdiopathic thrombocytopenic purpuraIgA nephropathyMultiple sclerosisPolyarteritis nodosaPolymyositisSarcoidosisTemporal arteritisBiochemical responseUrsodeoxycholic acidRisk-factorsCirrhosisPrognosisSarcoidosisManagementPBCGastroenterology & hepatologyAlkaline phosphataseAntibodies, antinuclearAspartate aminotransferasesAutoantibodiesBilirubinBiomarkersFemaleHumansLiver cirrhosis, biliaryMaleMitochondriaPrevalencePrognosisSex factorsAutoimmune diseasesArticle0005617067000012-s2.0-8508970681593694236432790935Gastroenterology & hepatologyCholangitis; Biliary Liver Cirrhosis; Obeticholic AcidAlkaline phosphataseAntinuclear antibodyAspartate aminotransferaseBilirubinMitochondrion antibodySmooth muscle antibodyAlkaline phosphataseAntinuclear antibodyAspartate aminotransferaseAutoantibodyBilirubinBiological markerAddison diseaseAdultAnkylosing spondylitisAntiphospholipid syndromeArticleAscitesAutoimmune diseaseAutoimmune hemolytic anemiaBleedingBullous pemphigoidCeliac diseaseChronic urticariaCohort analysisCollagenous colitisControlled studyCrohn diseaseDecompensated liver cirrhosisDermatomyositisEvent free survivalFemaleFollow upGastritisGraves diseaseHashimoto diseaseHepatic encephalopathyHumanIdiopathic thrombocytopenic purpuraImmunoglobulin A nephropathyInflammatory bowel diseaseLichen planusLichen sclerosus et atrophicusLiver cell carcinomaMajor clinical studyMaleMedical recordMembranous glomerulonephritisMiddle agedMultiple sclerosisMyasthenia gravisPemphigus vulgarisPernicious anemiaPolyarteritis nodosaPolymyositisPrevalencePrimary biliary cirrhosisRetrospective studyRheumatoid arthritisSarcoidosisSjoegren syndromeSystemic lupus erythematosusSystemic sclerosisTemporal arteritisUlcerative colitisUndifferentiated connective tissue diseaseVariceal bleedingVitiligoWegener granulomatosisAutoimmune diseaseBiliary cirrhosisBloodComplicationImmunologyMitochondrionPrevalencePrognosisSex factor