Abstract
Background
Inflammatory bowel diseases (IBD) and primary sclerosing cholangitis (PSC) are closely related. IBD-PSC has clinical characteristics that distinguish it from IBD without autoimmune liver disease (ALD). The association between autoimmune hepatitis (AH), overlapping syndrome (OS), primary biliary cirrhosis (PBC) and IBD is less known.
Methods
Clinical records of patients with IBD and PSC, AH, OS and PBC in a reference centre were reviewed retrospectively. Primary objective: to estimate the prevalence of ALD in patients with IBD. Secondary objectives: to establish the temporal gap between diagnosis of IBD and hepatopathy, severity of IBD, transplant requirement, displasia/colorectal carcinoma (CRC), and correlation between symptoms and endoscopic findings. All variables were compared according to liver disease.
Results
Of 1895 patients with IBD, 108 (5.7% [CI95% 4.7–6.8%]) had concomitant ALD, 78 PSC, 27 AH, 3 OS; no patient had PBC. 96.3% were associated with ulcerative colitis (UC). The prevalence of ALD in patients with UC was 7.6% (CI95% 6.3–9.2) and 1% (CI95% 0.3–2.5%) in patients with Crohn’s Disease. In patients with PSC, 56.4% were men, IBD diagnosis was previous than hepatopathy in 54.3% (median time 8.0 years), subsequent in 10% (7.9 years) and simultaneous, less than 1 year, in 35.7%. Conversely, in patients with AH, 55.6% were women, IBD was first diagnosed in 16% (median time 6.5 years), subsequent in 40% (5.3 years) and simultaneous in 44.0%. The median age at diagnosis was 32.5 (IQR 19.5–50.1) years for PSC and 13.1 (IQR 5.6–16.0) years for AH. Of 77 patients with available data, 97% had extensive colitis, without difference between PSC and AH. Patients with AH had more severe phenotype than PSC (Graphic 1).
High grade dysplasia/CRC was detected in 5 patients (9.6%) all with PSC. Liver transplantation was required in 38.5% of PSC-UC patients and in 20% of AH-UC patients. Regarding endoscopic-clinical concordance, 52% of patients with moderate-severe endoscopic score were asymptomatic with similar behaviour in both hepatopathies.
Conclusion
PSC and AH were the most frequent ALD associated with IBD, mostly extensive UC. In contrast to PSC, AH tended to be diagnosed before intestinal disease and at an earlier age with higher biological and hospital admissions requirement. More than a half of our cohort had an important clinical-endoscopic dissociation, so we emphasise the relevance of the objective measurement of inflammation with colonoscopy in this particular group of patients
Successful Treatment of Recurrent Primary Sclerosing Cholangitis after Orthotopic Liver Transplantation with Oral Vancomycin
