title: Non-IBD immunological diseases are a risk factor for reduced survival in PSC.
abstract: BACKGROUND
  Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. It is known to be associated with immunological diseases (IDs), such as inflammatory bowel disease (IBD) and autoimmune hepatitis (AIH).
  
  
  OBJECTIVE
  We evaluated the presence of IDs besides IBD and AIH in a cohort of PSC patients, and its association with clinical outcome.
  
  
  METHODS
  This is a prospective cohort study of 195 PSC patients that were evaluated over the period 1987-2010 in our tertiary care centre. The presence of ID was determined using a retrospective chart review. IDs were subclassified into autoimmune disease (AID) and immune-mediated inflammatory disease (IMID), according to current guidelines.
  
  
  RESULTS
  Twenty-seven of 195 (13.8%) PSC patients had at least one additional ID other than IBD (70%) or AIH (5%). The most frequent AIDs were autoimmune thyroiditis (2.6%) and diabetes mellitus type 1 (2.1%). The most frequent IMIDs were psoriasis (3.6%) and sarcoidosis (2.1%). After more than 20 years of follow-up, concomitant IDs represent an independent risk factor for reduced transplantation-free survival in patients with PSC (mean: 8.9 years vs. 16.3 years, P = 0.012). Further subgroup analysis revealed a significantly reduced survival especially in patients with concomitant IMID (P = 0.017).
  
  
  CONCLUSIONS
  Patients with concomitant IDs, especially IMID, are a clinically important subgroup of PSC patients. This significant phenotype warrants further genetic and immunological studies.
authors.affiliation: Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Rupp
authors.forename: Anne
authors.initials: A
authors.lastname: Mummelthei
authors.forename: Peter
authors.initials: P
authors.lastname: Sauer
authors.forename: Karl H
authors.initials: KH
authors.lastname: Weiss
authors.forename: Peter
authors.initials: P
authors.lastname: Schirmacher
authors.forename: Adolf
authors.initials: A
authors.lastname: Stiehl
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Stremmel
authors.forename: Daniel N
authors.initials: DN
authors.lastname: Gotthardt
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000401:mortality*; D016009:Chi-Square Distribution; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D015209:Cholangitis, Sclerosing / Q000401:mortality* / Q000628:therapy; D003922:Diabetes Mellitus, Type 1 / Q000401:mortality; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007249:Inflammation / Q000401:mortality*; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation; D008297:Male; D015999:Multivariate Analysis; D015995:Prevalence; D016016:Proportional Hazards Models; D011565:Psoriasis / Q000401:mortality; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012507:Sarcoidosis / Q000401:mortality; D062606:Tertiary Care Centers; D013967:Thyroiditis, Autoimmune / Q000401:mortality; D013997:Time Factors; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid