Centro Hospitalar de São João Porto, Porto, Portugal
Isabel Garrido, MD, Guilherme Macedo, PhD; Centro Hospitalar de São João, Porto, Porto, Portugal
Introduction: Primary biliary cholangitis (PBC) is a chronic autoimmune disease, characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. With the advent of treatment, the majority of patients have a life expectancy similar to that of the general population. However, without adequate vigilance and treatment, the disease can become potentially serious and patients may develop cirrhosis, hepatocellular carcinoma, metabolic bone disease and malabsorption. The aim of this study was to identify patients with PBC who were not adequately diagnosed and remained without proper treatment, as well as the clinical impact of loss to follow-up. Methods: We collected data retrospectively from adult patients with antimitochondrial antibodies (AMA) at a titer of 1:40 or higher between January 2010 and December 2019 at Centro Hospitalar e Universitário de São João, Portugal. A diagnosis of PBC was established if there was no extrahepatic biliary obstruction and at least one of the following were also present: elevated alkaline phosphatase or histologic evidence of PBC. Results: A total of 214 AMA-positive patients were included in the study, 148 (69.2%) of whom had diagnostic criteria for PBC. 124 patients were correctly diagnosed from the beginning and one individual was diagnosed and started treatment during the follow-up. However, there were 19 patients with diagnostic criteria for PBC right from the start that were not recognized, as well as four individuals who developed PBC during the follow-up and were not adequately followed and treated. Of these 23 patients who were lost to follow-up, most were females (69.6%) and with a median age of 59 years old (interquartile range 44-70 years). The majority of patients (69.6%) were followed by specialties other than Gastroenterology (Figure 1). There were 6 patients who developed cirrhosis and associated complications, two patients had been poor control of the underlying disease (autoimmune hepatitis) and one patient died of liver failure. Discussion: About 16% of patients with definitive diagnostic criteria for PBC were not detected, which hindered correct treatment and vigilance. Up to 39.1% of non-diagnosed patients developed complications related to liver disease which could be avoided with the proper etiological diagnosis. Thus, strategies must be established to identify these patients, especially when they are followed by other specialties.
Disclosures: Isabel Garrido indicated no relevant financial relationships. Guilherme Macedo indicated no relevant financial relationships.