Overlap syndrome with autoimmune hepatitis and primary biliary cholangitis

Authors

  • Harol Herrera Villalba Clínica General del Norte
  • Alcides Herrera Alvarado Clínica General del Norte
  • Fair Clavijo Tinoco Clínica General del Norte
  • Jorge Fernández Franco Clínica General del Norte
  • Ronald Maestre Serrano Universidad Libre
  • Mario Moscote Granadillo Clínica General del Norte

DOI:

https://doi.org/10.18041/2390-0512/biociencias.1.6366

Keywords:

Overlap syndrome, Autoimmune Hepatitis, Primary sclerosing cholangitis

Abstract

Introduction: The term "Overlap Syndrome" refers to one of the variant forms of autoimmune hepatitis, in which clinical and serological findings of autoimmune hepatitis are found in relation to the characteristics of certain cholestatic liver diseases such as primary biliary cholangitis (PBC) and Primary sclerosing cholangitis (PEC). Objetive: The early recognition of these variants is clinically significant from the point of view of treatment and prognosis. Methodology: A case is presented, corresponding to a female patient of 66 years of age who consulted for chronic clinical picture of generalized pruritus, and absence of dermatological lesions, with paraclinical studies that showed hypertransaminasemia and cholestatic pattern, with positivity for antimitochondrial, anti-smooth muscle and Inmunoglobulin G (IgG) with elevation of its concentrations. Conclusions: The histopathological study reported necroinflammatory activity in the absence of fibrous expansion, resulting in good prognosis, and instauration of therapeutic regimen, with UDCA, systemic corticosteroids, immunosuppressive agents, with adequate clinic response.

Downloads

Download data is not yet available.

References

Czaja AJ. Diagnosis and management of the overlap syndromes of autoimmune hepatitis. Canadian Journal of Gastroenterology. 2013;27(7):417-423.

2. Czaja AJ, et al. Cholestatic phenotypes of autoimmune hepatitis. Clin Gastroenterology and Hepatology. 2014; 12:1430–8.

3. Czaja Albert J, Herschel A. Carpenter. Autoimmune Hepatitis Overlap Syndromes and Liver Pathology. Gastroenterology Clinics of North America. 2017; Volume 46, Issue 2: Pages 345-364.

4. Chazouilleres O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology. 1998; 28:296-301.

5. Boberg, Kirsten Muri et al. Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. Journal of Hepatology. February 2011. Volume 54, Issue 2, Pages 374–385.

6. Beuers U, Boberg KM, Chapman RW, et al. EASL Clinical Practice Guidelines: management of cholestatic liver diseases. J Hepatol 2009; 51:237–67.

7. Kuiper EM, Zondervan PE, van Buuren HR. Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol. 2010; 8:530–4.

8. Gatselis NK, Zachou K, Papamichalis P, et al. Comparison of simplified score with the revised original score for the diagnosis of autoimmune hepatitis: ¿a new or a complementary diagnostic score? Dig Liver Dis 2010; 42:807–12.

9. Rust C, Beuers U. Overlap syndromes among autoimmune liver diseases. World J Gastroenterol 2008; 14(21): 3368-3373.

10. Hennes EM, Zeniya M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008; 48:169–76.

11. Vergani GM, Vergani D, Czaja AJ, Manns MP, Krawitt EL, Vierling JM, Lohse AW, Montano-Loza AJ. Autoimmune hepatitis. Nature Reviews Disease Primers. 2018; volume 4 article number 18017.

12. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Autoimmune hepatitis. J Hepatol. 2015; 63:971–1004.

13. Czaja. The role of autoantibodies as diagnostic markers of autoimmune hepatitis. Expert Rev Clin Immunol 2006; 2:33-48.

14. Montano-Loza AJ, Carpenter HA, Czaja AJ. Frequency, behavior, and prognostic implications of antimitochondrial antibodies in type 1 autoimmune hepatitis. J Clin Gastroenterol 2008; 42:1047–53.

15. Hu CJ, Zhang FC, Li YZ, Zhang X. Primary biliary cirrhosis: what do autoantibodies tell us? World J Gastroenterol. 2010; 16:3616–3629.

16. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB. Genetic predispositions for the immunological features of chronic active hepatitis. Hepatology 1993; 18:816.

17. Dorner T, Feis E, Held C, Conrad K, Burmester GR, Hiepe F. Differential recognition of the 52-kD Ro (SS-A) antigen by sera from patients with primary Sjögren’s syndrome. Hepatology 1996; 24:1404-7.

18. de Boer YS, van Nieuwkerk CM, Witte BI et al, Assessment of the histopathological key features in autoimmune hepatitis. Histopathology 2015; 66:351–362.

19. Ishak K, et al. Histological grading and staging of chronic hepatitis. J Hepatol. 1995;22(6):696.

20. Czaja AJ. Rapidity of treatment response and outcome in type 1 autoimmune hepatitis. J Hepatol 2009; 51:161–7.

Downloads

Published

2020-05-21

Issue

Section

CASE REPORTS

How to Cite

Overlap syndrome with autoimmune hepatitis and primary biliary cholangitis. (2020). Biociencias, 15(1), 103-111. https://doi.org/10.18041/2390-0512/biociencias.1.6366

Most read articles by the same author(s)