Analysis of the Clinical Course of Primary Sclerosing Cholangitis in Paediatric Population—Single Center Study

Background and Objectives: Primary sclerosing cholangitis (PSC) is a rare cholestatic disease of the liver of unknown etiology, severe course and poor prognosis. PSC most often co-occurs with inflammatory bowel diseases (IBD), especially with ulcerative colitis (UC). The aim of the study was the analysis of the clinical course of primary sclerosing cholangitis in children, hospitalized in the Gastroenterology Unit in Katowice. Materials and Methods: The analysis included 30 patients, aged from 7 to 18 years, 21/30 boys (70%) and 9/30 girls (30%), diagnosed with PSC in the years 2009–2019. The analysis included the age at diagnosis, clinical symptoms, course of the disease, coexisting diseases, laboratory and imaging results, and complications. Results: The average age at diagnosis was 13 years. 22/30 (73.3%) patients suffered from UC, 4/30 (13.3%) were diagnosed with Crohn’s disease (CD), 2/30 (6.66%) with Eosinophilic Colitis (EC). 2/30 patients (6.66%) had no clinical evidence of coexistent IBD to date. In addition, 7/30 (23.3%) had an overlap syndrome of primary sclerosing cholangitis/autoimmune hepatitis. When PSC was detected before IBD (6/30–20%), patients had complications more often compared to those diagnosed with IBD first or PSC and IBD at the same time. At the moment of diagnosis 6/30 (20%) patients presented with abdominal pain, which was the most common symptom, 3/30 (10%) jaundice, while 17/30 (56.6%) were asymptomatic but had abnormal results of the laboratory tests. Conclusions: Monitoring liver markers in IBD patients is important since most PSC cases are asymptomatic and their elevation might be the first sign of the disease. Patients diagnosed with PSC before IBD diagnosis are more likely to have a more aggressive course of the disease.

Difficulties in differential diagnosis of inflammatory bowel diseases in patient with overlapping autoimmune hepatitis and primary sclerosing cholangitis

The article presents a clinical case of cross-syndrome of autoimmune hepatitis type 1 with primary sclerosing cholangitis. The main symptoms, laboratory and instrumental criteria of these diseases are discussed. Detection of specific antibodies is necessary for an accurate diagnosis. Interpretation of changes in the colon obtained by stepwise biopsy requires special attention. The described morphological picture does not allow to make an accurate diagnosis, it can be regarded as initial manifestations of Crohn's disease, microscopic or eosinophilic colitis, signs of worm-parasitic invasion. An example of a differential diagnostic search in such a situation is discussed.