The Serological Profile of the Autoimmune Hepatitis/Primary Biliary Cirrhosis Overlap Syndrome

Muratori, Paolo; Granito, Alessandro; Pappas, Georgios; Pendino, Gaspare M.; Quarneti, Chiara; Cicola, Ronny; Menichella, Rita; Ferri, Silvia; Cassani, Fabio; Bianchi, Francesco B.; Lenzi, Marco; Muratori, Luigi
June 2009
American Journal of Gastroenterology;Jun2009, Vol. 104 Issue 6, p1420
Academic Journal
OBJECTIVES:During the last decade patients with concomitant clinical, biochemical, immunoserological, and histological features of both autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) were sporadically described, but definite diagnostic criteria and specific serological markers to support the diagnosis of AIH/PBC overlap syndrome (AIH/PBC OS) are still lacking.METHODS:Clinical, biochemical, and histological features, autoantibody profile, and treatment response of 15 patients with coexistent hepatitic and cholestatic liver damage, all fulfilling strict diagnostic criteria for both AIH and PBC, were compared with those of 120 patients with pure PBC and 120 patients with pure AIH.RESULTS:At diagnosis, the AIH/PBC OS patients' median age was 51 years, similar to that of the PBC patients (52 years, P=NS), but significantly higher than that of the AIH patients (40 years, P=0.04). Anti-dsDNA antibodies were detected in 60% of AIH/PBC OS patients, but only in 4% of PBC patients and 26% of AIH patients (P<0.0001 and 0.01, respectively). Double positivity for antimitochondrial antibodies (AMA) and anti-dsDNA was present in 47% of those with AIH/PBC OS, but only in 2% of the pathological controls (P<0.0001; specificity: 98; 95% confidence interval (CI): 97–99.2; positive likelihood ratio: 28; 95% CI: 9.8–79.4). Combined therapy (ursodeoxycholic acid (UDCA) plus steroids) achieved biochemical response in 77% of AIH/PBC OS patients.CONCLUSIONS:Concomitant AMA/anti-dsDNA seropositivity can be considered the serological profile of AIH/PBC OS. The combination of UDCA and steroids is effective in achieving persistent biochemical amelioration in most AIH/PBC OS patients.Am J Gastroenterol 2009; 104:1420–1425; doi:10.1038/ajg.2009.126; published online 28 April 2009


Related Articles

  • Challenges in Diagnosing Hepatic Encephalopathy. Weissenborn, K. // Neurochemical Research;Feb2015, Vol. 40 Issue 2, p265 

    The term 'hepatic encephalopathy' (HE) covers the neuropsychiatric syndrome associated with acute, chronic and acute-on-chronic liver disease (CLD). This paper deals with clinical features and diagnosis of HE in patients with liver cirrhosis and portal hypertension or porto-systemic shunts. The...

  • Evaluation of cross-reactive antibody response to HVR1 in chronic hepatitis C. Bing-Shui Xiu; Xiao-Yan Feng; Jing He; Guo-Hua Wang; Xiang-Ying Zhang; He-Qiu Zhang; Xiao-Guo Song; Kun Chen; Shi-Gan Ling; Cui-Xia Zhu; Lai Wei; Hui-Ying Rao // Annals of Gastroenterology & Hepatology;2010, Vol. 8 Issue 89, p4460 

    AIM: To evaluate the presence and cross-reactive antibodies against hypervariable region 1 (HVR1) in hepatitis C virus (HCV) infected patients and its relationship with the progression of the disease. METHODS: Sixteen representative HVR1 proteins selected from a unique set of 1600 natural...

  • Value of computerized inhibitory control test and blood tests in minimal hepatic encephalopathy diagnosis. Savlan, Ilona; Liakina, Valentina; Valantinas, Jonas // Acta Medica Lituanica;2013, Vol. 20 Issue 3, p109 

    Background. Minimal hepatic encephalopathy (MHE) can be diagnosed by "paper-pencil" tests, computerised inhibitory control or critical flicker frequency tests, but for clinical practice more convenient methods of diagnosis are being searched. The aim of the study was to assess the value of...

  • Missed Opportunities for Hepatocellular Carcinoma Screening in an HIV/Hepatitis C Virus–Coinfected Cohort. Beauchamp, Elizabeth; Rollet, Kathleen; Walmsley, Sharon; Wong, David K.; Cooper, Curtis; Klein, Marina B. // Clinical Infectious Diseases;Nov2013, Vol. 57 Issue 9, p1339 

    We examined adherence to guidelines for screening of hepatocellular carcinoma in a cohort of HIV/hepatitis C virus–coinfected patients. Thirty-six percent of patients with documented cirrhosis did not have a screening ultrasound. Patients at centers with standardized systems for screening...

  • Diagnosis and Prognostic Significance of Minimal Hepatic Encephalopathy in Patients with Cirrhosis of Liver. Dhiman, Radha; Kurmi, Roshan; Thumburu, Kiran; Venkataramarao, Sunil; Agarwal, Ritesh; Duseja, Ajay; Chawla, Yogesh // Digestive Diseases & Sciences;Aug2010, Vol. 55 Issue 8, p2381 

    Minimal hepatic encephalopathy is the mildest form of the spectrum of hepatic encephalopathy (HE) that impairs health-related quality of life. We assessed (1) the usefulness of psychometric hepatic encephalopathy score and critical flicker frequency for the diagnosis of minimal hepatic...

  • Progression of liver stiffness predicts clinical events in HIV/HCV-coinfected patients with compensated cirrhosis. Merchante, Nicolás; Téllez, Francisco; Rivero-Juárez, Antonio; Ríos-Villegas, Maria José; Merino, Dolores; Márquez-Solero, Manuel; Omar, Mohamed; Recio, Eva; Pérez-Pérez, Montserrat; Camacho, Ángela; Macías-Dorado, Sara; Macías, Juan; Lorenzo-Moncada, Sandra; Rivero, Antonio; Pineda, Juan A.; Grupo Andaluz para el Estudio de las Hepatitis Víricas (HEPAVIR) de la Sociedad Andaluza de Enfermedades Infecciosas (SAEI) // BMC Infectious Diseases;12/7/2015, Vol. 15, p1 

    Background: Our objective was to assess the predictive value of the changes of liver stiffness (LS) for clinical outcome in HIV/HCV-coinfected patients with compensated liver cirrhosis and a LS value < 40 kPa.Methods: Prospective cohort of 275 HIV/HCV-coinfected...

  • Diagnosis of minimal hepatic encephalopathy in a tertiary care center from eastern Romania: validation of the psychometric hepatic encephalopathy score (PHES). Badea, Mircea; Drug, Vasile; Dranga, Mihaela; Gavrilescu, Otilia; Stefanescu, Gabriela; Popa, Iolanda; Mihai, Catalina; Cijevschi-Prelipcean, Cristina // Metabolic Brain Disease;Dec2016, Vol. 31 Issue 6, p1463 

    The psychometric hepatic encephalopathy score (PHES) is frequently used as a 'gold standard' for the diagnosis of minimal hepatic encephalopathy (MHE). In Romania, there are currently no widely available tests for the detection of MHE. In this study we aimed to standardize the PHES in a healthy...

  • Qualifying and quantifying minimal hepatic encephalopathy. Morgan, Marsha; Amodio, Piero; Cook, Nicola; Jackson, Clive; Kircheis, Gerald; Lauridsen, Mette; Montagnese, Sara; Schiff, Sami; Weissenborn, Karin // Metabolic Brain Disease;Dec2016, Vol. 31 Issue 6, p1217 

    Minimal hepatic encephalopathy is the term applied to the neuropsychiatric status of patients with cirrhosis who are unimpaired on clinical examination but show alterations in neuropsychological tests exploring psychomotor speed/executive function and/or in neurophysiological variables. There is...

  • Quantitative magnetic resonance imaging in patients with cirrhosis: a cross-sectional study. Grover, Vijay; Crossey, Mary; Fitzpatrick, Julie; Saxby, Brian; Shaw, Roberta; Waldman, Adam; Morgan, Marsha; Taylor-Robinson, Simon // Metabolic Brain Disease;Dec2016, Vol. 31 Issue 6, p1315 

    Cerebral magnetic resonance imaging was undertaken, at 3 Tesla field strength, employing magnetization transfer (MT) and diffusion-weighted imaging (DWI) sequences, in 26 patients with well-compensated cirrhosis, free of overt hepatic encephalopathy. Results were compared to those from 18...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics