Primary biliary cirrhosis: a retrospective study of 36 cases

##plugins.themes.academic_pro.article.sidebar##

Published Apr 2, 2014
Download
pdf
Vol. 92 No. 2 (2014): Issue Feb 2014

##plugins.themes.academic_pro.article.main##

Mehdi Ksiaa
Mohamed Ajili
Aida.Ben Slama
Ahlem. Souguir
Mohamed.Ben Rjeb
Badreddine Sriha
Ahlem Brahem
Ali Jmaa
Salem Ajmi

Abstract

Background: Primary biliary cirrhosis is a rare cholestasis liver disease affecting the women of mature age,it association with Autoimmune hepatitis defined the overlap syndrome.
aim : It was to determine epidemiological, clinic-biological, immunological, histological, therapeutic and evolutive characteristics of PBC and to compare them to those in subjects having an overlap syndrome.
methods: It is a retrospective study grouping all the cases of PBC hospitalized over a period of 15 years (1995-2009) in the hepatogastro-enterology department at Sahloul hospital in Sousse.
results: Thirty six cases were grouped over the period of the study. They were all women. The mean age was 54.5 years (28-79). The disease was symptomatic at the moment of diagnosis in 75% of the cases. The functional signs revealing the disease were principally asthenia, present in 69.4% of the cases, followed by jaundice and prurit. Cholestasis was constant in all the patients, stage III of scheuer was the most frequent in the patients (25%) followed by stage IV (22%). OS was present in 1/3 of the patients; the treatment is based on the prescription of urso-desoxycholic Acid associated with corticoids and with immunosuppressors in the case of OS. The average survival of the patient having PBC and OS was comparable. It was respectively of 61.8 and 55.9 months.
Conclusion: Our results as well as the literature reveal the rarety of this disease and its predominance in women. PBC has to be diagnosed at an early stage to guarantee a better response and a better survival of the patients.

Keywords:

Hepatology - Primary biliary cirrhosis - Anti-mitochondrial antibody - Overlap syndrome - Urso-desoxy-cholic acid.

##plugins.themes.academic_pro.article.details##

References

  1. Raoul Poupon. Primary biliary cirrhosis: A 2010 update.Journal of hepathology 2010; 80: 14.
  2. Debbech R, Maamouri N, Ajmi S, Azzouz M et al. L'hépatite auto-Immune en Tunisie. Etude multicentrique rétrospective de 83 cas. La Tunisie Médicale 2010 ; Vol88 (n°11) :834-840.
  3. Prince M, Chetwyn A, Craig W, Metcalf J et James O. Asymptomatic primary biliary cirrhosis: clinical feature, prognosis and symptom progression in a large population based cohort. Gut 2004; 53: 865-70.
  4. Fox RA, Scheuer PJ, Scherlock S et al. Asymptomatic PBC. Gut 1973; 14: 444-7.
  5. Springer J, Cauch-Dudek K, Orourke K, Wanless IR et Heathcote J. Asymptomatic primary biliary cirrhosis: A study of naturel history and prognosis. Am J Gasteroenterol 1999; 94: 47-53.
  6. Lucey MR, Neuberger J et Williams R. Primary biliary cirrhosis in men. Gut 1986; 27: 1373-6.
  7. Jones EA et Bergasa NV. The pruritus of cholestasis. Hepatotogy 1999; 29: 1003-6.
  8. Neuberger J et Thomas R. PBC and AMA -What is the connection? Hepatology 1999; 29: 271-6.
  9. Munoz LE, Thomas HC, Scheuer PJ, Doniach D et Sherlock S. Is mitochondrial antibody diagnostic of primary biliary cirrhosis? Gut 1981; 22: 136-40.
  10. Klein R, Pointner H, Zilly W et al. Antimitochondrial antibody profiles in primary biliary cirrhosis distinguish at early stages between a benign and a progressive course: a prospective study on 200 patients followed for 10 years. Liver 1997; 17.
  11. Joshi S, Cauch-Dudek K, Heathocote J, Lindor K, Jorgensen R et Klein R. Antimitochondrial antibody profiles: Are they valid prognostic indicators in primary biliary cirrhosis ?A m J Gasteroenterol 2002; 97: 999-1002
  12. Talwalker JA et Lindor KD. Primary biliary cirrhosis. Lancet 2003; 362: 53-8.
  13. Kaplan MM. Primary biliary cirrhosis. N Engl J Med 1987; 316: 521-8.
  14. Kumagi T et Heathcote EJ. Primary biliary cirrhosis. Orphanet journal of rare diseases 2008; 3: 1750-67.
  15. 1J scheuer P. Primary biliary cirrhosis. Proc. R.Soc. med 1967;60:1257-60.
  16. Heurgué A, Vitry F, Diebold MD et al. Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis: a retrospective study of 115cases of autoimmune liver disease. Gasteroenterologie Clinique et Biologique. 2007; 31(1): 17-9.
  17. Roll J, Boyer J, Barry D et Klatskin G. The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 1983; 308: 1-7.
  18. Huet P, Deslauriers J, Tran A, Faucher C et Charbonneau. Impact of fatigue on the quality of life of patients with primary biliary cirrhosis. Am J Gastroenterol 2000; 95: 760-7.
  19. Christensen. J Hepatology 1999; 30: 285-92.
  20. Roll J, Boyer J, Barry D et Klatskin G. The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 1983; 308: 1-7.
  21. Corpechot C, Carrat F, Bonnand AM, Poupon R E et Poupon R. The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis. Hepatology 2000; 32: 1196-9.