Stancescu, Iulia, Rusu, Ioana, Pojoga, Cristina, Rednic, Voicu, Mercea, Voicu, Claudia, Buzas, Munteanu, Lidia, Petrusev, Bobe, Zaharie, Toader, Socaciu, Mihai, and Seicean, Andrada
Background. Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic disease that is associated with inflammatory bowel disease (IBD) in approximately 70-75% of cases. Most cases of IBD are represented by ulcerative colitis (UC) (80%). Existing data are from the literature, but the profile of this rare disease in association with inflammatory bowel disease in Romania is less studied. Aims. The clinic-demographic profile in patients diagnosed with sclerosing cholangitis. Methods. We performed a retrospective analysis of patients diagnosed with PSC between Jan 2018 to Oct 2022 in a tertiary medical center. The diagnosis was based on both typical aspect on MRI (paucity of biliary ducts with dilatations and stenosis) and hepatic biopsy. All patients had colonoscopy with ileal intubation with biopsy for excluding the association of inflammatory bowel diseases. Results. From 14 patients diagnosed with PSC there were 11 patients included, mean age was 46, male: female ratio being 3:2. UC was identified in 89% of cases, as pancolitis in 4 (36%) patients. Only one patient was diagnosed with ileo-colonic Crohn's disease (CD). The clinical presentation included obstructive jaundice in 5 (45%) patients, liver cirrhosis in 3 (27%) patients (Child-Pugh B in all cases) and cholangiocarcinoma in one (9%) patient. The MRCP aspect showed a dominant stricture in 5 (45%) patients, located in the hilum in 2 (18%) cases. Perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) were not suggestive for diagnosis, were identified in only 9% of cases. ERCP with stenting was performed in case of dominant strictures with cholangitis in 5 (45%) patients, with secondary lithiasis in 2 (18%) cases. Conclusions. The association of PSC-inflammatory bowel disease is a rare disease, with involvement of ulcerative colitis in most of the patients and mild symptoms. [ABSTRACT FROM AUTHOR]