Characteristics of patients with overlap syndrome of autoimmune liver diseases in a third level hospital.

Introduction and Objectives: Autoimmune liver disease is classified into 3 well-defined entities: autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis, there is a group of patients who show characteristics of more than one entity and is called overlap syndrome (OS), a...

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Main Authors: Orestes J. Cobos-Quevedo, Ximena E. Cuenca Avila, Viridiana López-Ladrón de Guevara, José L. Pérez-Hernández, María F Higuera-de la Tijera
Format: Article
Language:English
Published: Elsevier 2024-02-01
Series:Annals of Hepatology
Online Access:http://www.sciencedirect.com/science/article/pii/S1665268124002436
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Summary:Introduction and Objectives: Autoimmune liver disease is classified into 3 well-defined entities: autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis, there is a group of patients who show characteristics of more than one entity and is called overlap syndrome (OS), according to the Paris criteria they are classified, the prevalence of overlap in our country is low. We describe the clinical and biochemical characteristics of patients with OS treated at the liver clinic at the Hospital General de México. Materials and Patients: It is a retrospective and descriptive study, records of the autoimmune liver disease consultation were reviewed, searching for patients with OS using the Paris criteria in the period 2014-2023, descriptive statistics were performed with measures of central tendency and dispersion using SPSS 25.0. Results: 22 patients were included, all of them with liver biopsy, 95% women aged 47 ± 12.6 years, the most common phenotype was PBC/HAI (59%). The time to diagnosis from initial manifestations ranged from 1 to 6 years, the most frequent tests were ANA (81%), AMA (63%), ASMA (18%) and LKM1 (18%), Immunoglobulin G levels on average 2048 ±643.8. The most frequent comorbidities were systemic sclerosis, arterial hypertension and hypothyroidism, the predominant symptoms were fatigue and pruritus reported in 36%; 90% were cirrhotic, Child Pugh A 70%, B 25% and C 5%. The most frequent decompensation was variceal hemorrhage (22.7%), 4.7% reported portal thrombosis, and 2 patients were transplanted. Conclusions: Overlap syndromes are rare, we found the majority of patients are women with advancend stage of liver disease, the most frequent overlapping is PBC/HAI with a high proportion of positive serology tests and concordant biopsy, two patients underwent liver transplantation. It is important to always look for this rare syndrome.
ISSN:1665-2681