Laetitia Nguyen1, Nora Cazzagon2,3, Christophe Corpechot2, Sanaâ El Mouhadi1, Sara Lemoinne2, Olivier Chazouillères2, Lionel Arrivé4,5. 1. Faculté de Médecine Pierre et Marie Curie and Department of Radiology, Saint-Antoine Hospital, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France. 2. Hepatology Department, Reference Center for Inflammatory Biliary Diseases and Autoimmune Hepatitis, French Network for Rare Liver Diseases in Adults and Children (FILFOIE), Saint-Antoine Hospital, Assistance Publique - Hôpitaux de Paris (APHP), INSERM, UMR_S 938, CDR Saint-Antoine, UPMC Univ Paris 06, Paris, France. 3. Department of Surgery, Oncology and Gastroenterology, University of Padua, Padova, Italy. 4. Faculté de Médecine Pierre et Marie Curie and Department of Radiology, Saint-Antoine Hospital, Assistance Publique-Hôpitaux de Paris, Sorbonne Université, Paris, France. lionel.arrive@aphp.fr. 5. Department of Radiology, Saint-Antoine Hospital, 184 Rue du Faubourg Saint-Antoine, 75012, Paris, France. lionel.arrive@aphp.fr.
Abstract
AIMS: To evaluate the prognostic value of cystic dilatation (CD) of the intrahepatic biliary ducts in patients with primary sclerosing cholangitis (PSC). METHODS: A single-center cohort of 205 patients with PSC from 2003 to 2016 was analysed. CD was defined by quantitative and qualitative criteria. Radiological and clinical courses were assessed. A Kaplan-Meier analysis was used to estimate cumulative survival without liver transplantation (LT) from the date of PSC diagnosis. A log-rank test was performed to compare survival time of PSC patients with and without CD. RESULTS: A total of 15 (7.3%) PSC patients (12 males) with a median age of 23 years at diagnosis had CD. Five patients had one CD; seven patients had two or three CDs; and three patients had diffuse CD. CDs ranged in small diameter size from 12 to 32 mm. Radiological evolution of CD was markedly variable. However, a radiological worsening of PSC over time was observed in all patients. The clinical course was characterized by the occurrence of complications in most patients. Half of the patients with CD underwent LT at a median time of 40 months from diagnosis of CD and the median survival time from PSC diagnosis was significantly lower than in PSC without CD (10.7 vs. 23.4 years; HR 3.8, 95% confidence interval: 1.7-8.3, p = 0.001). CONCLUSIONS: CD in PSC is an unusual condition that mostly affects young patients. It is characterized by a rapid, unfavorable course and constitutes a significant prognostic factor. KEY POINTS: • Cystic dilatation of the intrahepatic biliary ducts affects young patients with primary sclerosing cholangitis and is characterized by a markedly variable radiological evolution. • Biliary wall inflammation, found in explanted livers, could be a key feature in the pathogenesis of cystic dilatation. • Cystic dilatation of the intrahepatic biliary ducts is characterized by an unfavorable course and constitutes a significant prognostic factor of primary sclerosing cholangitis.
AIMS: To evaluate the prognostic value of cystic dilatation (CD) of the intrahepatic biliary ducts in patients with primary sclerosing cholangitis (PSC). METHODS: A single-center cohort of 205 patients with PSC from 2003 to 2016 was analysed. CD was defined by quantitative and qualitative criteria. Radiological and clinical courses were assessed. A Kaplan-Meier analysis was used to estimate cumulative survival without liver transplantation (LT) from the date of PSC diagnosis. A log-rank test was performed to compare survival time of PSC patients with and without CD. RESULTS: A total of 15 (7.3%) PSC patients (12 males) with a median age of 23 years at diagnosis had CD. Five patients had one CD; seven patients had two or three CDs; and three patients had diffuse CD. CDs ranged in small diameter size from 12 to 32 mm. Radiological evolution of CD was markedly variable. However, a radiological worsening of PSC over time was observed in all patients. The clinical course was characterized by the occurrence of complications in most patients. Half of the patients with CD underwent LT at a median time of 40 months from diagnosis of CD and the median survival time from PSC diagnosis was significantly lower than in PSC without CD (10.7 vs. 23.4 years; HR 3.8, 95% confidence interval: 1.7-8.3, p = 0.001). CONCLUSIONS:CD in PSC is an unusual condition that mostly affects young patients. It is characterized by a rapid, unfavorable course and constitutes a significant prognostic factor. KEY POINTS: • Cystic dilatation of the intrahepatic biliary ducts affects young patients with primary sclerosing cholangitis and is characterized by a markedly variable radiological evolution. • Biliary wall inflammation, found in explanted livers, could be a key feature in the pathogenesis of cystic dilatation. • Cystic dilatation of the intrahepatic biliary ducts is characterized by an unfavorable course and constitutes a significant prognostic factor of primary sclerosing cholangitis.
Entities:
Keywords:
Cholangiography; Cholangitis; Magnetic resonance imaging; sclerosing
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