In:
Liver International, Wiley, Vol. 33, No. 1 ( 2013-01), p. 86-93
Abstract:
Primary sclerosing cholangitis ( PSC ) is a chronic cholestatic liver disease. It is known to be associated with immunological diseases ( ID s), such as inflammatory bowel disease ( IBD ) and autoimmune hepatitis ( AIH ). Aim We evaluated the presence of ID s 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. ID s 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 AID s were autoimmune thyroiditis (2.6%) and diabetes mellitus type 1 (2.1%). The most frequent IMID s were psoriasis (3.6%) and sarcoidosis (2.1%). After more than 20 years of follow‐up, concomitant ID s 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). Conclusion Patients with concomitant ID s, especially IMID , are a clinically important subgroup of PSC patients. This significant phenotype warrants further genetic and immunological studies.
Type of Medium:
Online Resource
ISSN:
1478-3223
,
1478-3231
DOI:
10.1111/liv.2012.33.issue-1
Language:
English
Publisher:
Wiley
Publication Date:
2013
detail.hit.zdb_id:
2102783-3
detail.hit.zdb_id:
2124684-1
