Jegadeesan, Ramprasad, Navaneethan, Udayakumar, Bharadwaj, Shishira, Hammel, Jeffrey, Sanaka, Madhusudhan R., Shen, Bo
funding text
Dr. Bo Shen is supported by the Ed and Joey Story Endowed Chair.
abstract
Background: The association between primary sclerosing cholangitis (PSC) and underlying inflammatory bowel disease (IBD) is well established. There are scant data on the association between non-IBD immunological diseases (NID) and PSC outcomes. Our objective was to investigate the impact of NID on the clinical outcomes in patients with PSC. Methods: We included 287 patients with PSC from 1985 to 2013 from our tertiary care data registry. Univariate and multivariate analyses were performed to assess the risk factors for liver transplantation. Results: Of the 287 patients with PSC, 38 (13.2%) patients had at least 1 concomitant immunological disease other than IBD; 241 patients (84.0%) had concurrent IBD. The most frequent NIDs were autoimmune thyroiditis, autoimmune hepatitis, and rheumatoid arthritis. The median follow-up time did not differ significantly between PSC patients with and without NID (10.5 years versus 7.0 years, P = 0.04). We did not find significant difference in the median time from PSC diagnosis to liver transplantation between PSC patients with and without NID (5.2 versus 6.3 years, P = 0.74). In the subgroup analysis, there was no significant difference in the median time from PSC diagnosis to liver transplantation between the PSC-only group, PSC with IBD group, and PSC with NID group (5.4 versus 6.4 versus 5.2 years, P = 0.22). Conclusions: The association of NID in patients with PSC did not seem to affect the need for liver transplantation or transplantation-free survival. The findings suggest that the increased load of autoimmunity, including the presence of IBD or NID, has a minimum impact on the disease outcome of PSC.