Clinical characteristics of 13 patients with primary sclerosing cholangitis

Abstract

Abstract: Objective To investigate the clinical characteristics of patients with primary sclerosing cholangitis (PSC) to enhance understanding of the disease and reduce misdiagnosis and missed diagnoses. Methods A retrospective analysis was conducted on the clinical characteristics, diagnostic methods, treatment approaches, and prognosis of patients diagnosed with PSC at the Second Affiliated Hospital of Kunming Medical University from January 2013 to November 2022. Results Of the 13 patients, 8 (66.7%) were male, with a mean age of 46.1 years and a disease duration of 3 (1~26.5) months, Large-duct PSC was diagnosed in 9 patients (69.2%) . Twekve patients presented with symptoms, while one was asymptomatic due to hepatic insufficiency. Key symptoms included abdominal pain in 9 patients (75.0%), pruritus in 3 (25.0%), jaundice in 7 (58.3%), and fatigue in 5 (41.7%). One patients had concurrent primary biliary cholangitis, two had chronic pancreatitis, and three (23.1%) had ulcerative colitis. Liver biochemical abnormalities, including elevated liver enzymes, hyperbilirubinemia, and hypoproteinemia, were present in all patients. Immunological testing revealed ANA positivity in 60%(6/10) of patients and pANCA positivity in 25%. MRCP findings indicated bile duct wall irregularities, including uneven thickness, rigid courses, partial strictures and dilatations, and a “withered tree-like” appearance in 2 cases. Of the 10 patients who underwent liver biopsy, only one displayed the characteristic “Onion skin-like” fibrosis. All 13 patients were treated with ursodeoxycholic acid; 2 also received hormone therapy, and 2 showed limited response to treatment. The median follow-up duration was 41 months (1-86 months), with one patient lost to follow-up and one death due to acute liver failure. Conclusion PSC primarily affects middle-aged men and predominatly involves large-duct disease. Its clinical manifestations are nonspecific, frequently accompanied by ulcerative colitis, and may co-occur with other autoimmune liver diseases. Cholangiography remains the primary diagnostic tool, while treatment with ursodeoxycholic acid can improve clinical outcomes.

Key words: Primary sclerosing cholangitis, Diagnosis, Therapy, Prognosis

XIA Shu-min, TANG Ying-mei, XU Jia-min, ZHU bi-lian, LI Qin-nian. Clinical characteristics of 13 patients with primary sclerosing cholangitis[J]. Chinese Hepatolgy, 2024, 29(11): 1396-1400.

References

[1] Dyson J K, Beuers U, Jones D E J, et al. Primary sclerosing cholangitis[J]. Lancet, 2018, 391(10139): 2547-2559.
[2] Pj T, Cl B, Kk Y, et al. Epidemiology, natural history, and outcomes of primary sclerosing cholangitis: a systematic review of population-based studies[J]. Clin Gastroenterol Hepatol, 2022, 20(8): 1687-1700.
[3] 中华医学会肝病学分会.原发性硬化性胆管炎诊断及治疗指南(2021)[J]. 临床肝胆病杂志,2022,38(01):50-61.
[4] Chapman M H, Thorburn D, Hirschfield G M, et al. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. [J]. Gut, 2019, 68(8): 1356-1378.
[5] Tan N, Ngu N, Worland T, et al. Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study[J]. Hepatol Int, 2022, 16(5): 1094-1104.
[6] Lindkvist B, Benito De Valle M, Gullberg B, et al. Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in sweden[J]. Hepatology, 2010, 52(2): 571-577.
[7] Shneider B L. Diagnostic and therapeutic challenges in pediatric primary sclerosing cholangitis[J]. Liver Transpl, 2012, 18(3): 277-281.
[8] Stevens J P, Gupta N A. Recent insights into pediatric primary sclerosing cholangitis[J]. Clin Liver Dis, 2022, 26(3): 489-519.
[9] 中华医学会肝病学分会.自身免疫性肝炎诊断和治疗指南(2021)[J]. 临床肝胆病杂志, 2022,38(1):42-49.
[10] Björnsson E, Boberg K M, Cullen S, et al. Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis[J]. Gut, 2002, 51(5): 731-735.
[11] Ringe K I, Bergquist A, Lenzen H, et al. Clinical features and MRI progression of small duct primary sclerosing cholangitis (PSC). [J]. Eur J Radiol, 2020, 129: 109101.
[12] 苗琪,陈晓宇.自身免疫性肝病相关病理特征[J].胃肠病学, 2018, 23(5): 287-292.
[13] Sarcognato S, Sacchi D, Grillo F, et al. Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis[J]. Pathologica, 2021, 113(3): 170-184.
[14] Mendes F D, Jorgensen R, Keach J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. [J]. Am J Gastroenterol, 2006, 101(9): 2070-2075.
[15] Berstad A E, Aabakken L, Smith H-J, et al. Diagnostic accuracy of magnetic resonance and endoscopic retrograde cholangiography in primary sclerosing cholangitis[J]. Clin Gastroenterol Hepatol, 2006, 4(4): 514-520.
[16] Zhang Y, Gao X, He Z, et al. Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: a systematic review and meta-analysis[J]. Liver Int, 2022, 42(8): 1814-1822.
[17] Loftus E V, Harewood G C, Loftus C G, et al. PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis [J]. Gut, 2005, 54(1): 91-96.
[18] Mitchell S A, Bansi D S, Hunt N, et al. A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis[J]. Gastroenterology, 2001, 121(4): 900-907.
[19] Lindor K D, Kowdley K V, Luketic V A C, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis[J]. Hepatology.2009,50(3):808-814.
[20] European Association For The Study Of The Liver. EASL clinical practice guidelines on sclerosing cholangitis[J]. J Hepatol, 2022, 77(3): 761-806.
[21] Aabakken L, Karlsen T H, Albert J, et al. Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline. [J]. J Hepatol, 2017, 66(6): 1265-1281.
[22] Tabibian J H, Baron T H. Endoscopic management of primary sclerosing cholangitis[J]. Expert Rev Gastroenterol Hepatol, 2018, 12(7): 693-703.
[23] Weismüller T J, Trivedi P J, Bergquist A, et al. Patient age, sex, and inflammatory bowel disease phenotype associate with course of primary sclerosing cholangitis[J]. Gastroenterology, 2017, 152(8): 1975-1984.