Clinicobiological and morphological profiles of primary sclerosing cholangitis in tunisia

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Published Sep 15, 2014
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Vol. 92 No. 5 (2014): Issue May 2014

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Héla Kchir
Asma Ouaka Kchaou
Ali Amouri
Nadia Ben Mahmoud
Mohamed Moussadek Azzouz
Nabil Tahri
Department of gastroenterology University Hospital Hedi Chaker Sfax Tunisia
Abdeljabbar Ghorbel
Béchir Zouari
Nabyl Ben Mami

Abstract

Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease strongly associated, in the western series, to inflammatory bowel disease (IBD) and particularly to chronic ulcerative colitis. North African data are rare.
aim : To study the epidemiological, clinicobiological and morphological profile of PSC in Tunisia.
methods: A retrospective multicenter study extended over a period of 14 years (1995-2009), including all patients suffering from PSC, hospitalized in the four participants departments. We collected epidemiological, clinico-biological, histopathologic and morphological data for each patient.
results: We brought together 33 patients (22 men and 11 women), middle aged 44 years. The disease was symptomatic in 73% of cases. Laboratory tests showed cholestasis (100%) associated with hyperbilirubinemia (72%) and a moderate cytolysis (78%). Morphological analysis of bile ducts by cholangioMRI or endoscopic retrograde cholangio-pancreatography objectified diffuse damage of the biliary tract in 61% of cases. Association with IBD was found in 33% of cases (Crohn's Disease: 27%, chronic ulcerative colitis: 6%). An association with autoimmune hepatitis was found in 6% of cases and primary biliary cirrhosis in 3% of cases.
Conclusion : PSC is rare in Tunisia and affects men more often than women. The association with IBD is less frequent than in literature. It concerns essentially Crohn's disease. These data require confirmation by prospective multicenter studies.

Keywords:

Primary sclerosing cholangitis, biliary ducts, inflammatory bowel disease

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References

  1. Abdalian R, Heathcote EJ. Sclerosing cholangitis: a focus on secondary causes. Hepatology 2006;44:1063-74.
  2. Chazouillères O, Housset C. Intrahepatic cholestasis. Textbook of hepatology: from basic science to clinical practice 3e. Oxford: Blackwell Publishing, 2007.
  3. Smith MP, Loe RH. Sclerosing cholangitis; Review of recent case reports and associated diseases and four new cases. Am JSurg 1965; 110: 239-46.
  4. Abdalian R, Heathcote EJ. Sclerosing cholangitis: a focus on secondary causes. Hepatology 2006; 44:1063-74
  5. Majoie C, Reeders J, Sanders JB, Huibergtse K, Jansen P, Primary sclerosing cholangites: A modified classification of cholangiographic findings AJR 1991 157: 495-7.
  6. Ludwig J, Barham SS, Larusso NF, Elveback LR, Wiesner RH, McCall JT. Morphologic features of chronic hepatitis associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hepatology 1981; 6: 632-40.
  7. Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 1998; 33: 99-103.
  8. Bambha K, Kim WR, Talwalkar J, et al. Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community. Gastroenterology 2003;125:1364-9.
  9. Takikawa H, Takamori Y, Tanaka A, Kurihara H, Nakanuma Y. Analysis of 388 cases of primary sclerosing cholangitis in Japan; presence of a subgroup without pancreatic involvement in older patients. Hepatol Res 2004;29: 153-9.
  10. Talwalkar JA, Lindor KD. Primary sclerosing cholangitis. Inflamm Bowel Dis 2005;11:62-72.
  11. Geonzon-Gonzales MR. Primary sclerosing cholangitis. Gastroenterology nursing 2007;30:102-5.
  12. Lee Y.M, Kaplan M. Primary sclerosing cholangitis. N Engl J Med 1995;332:925-33.
  13. Wiesner RH, Larusso NF. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology 1980; 79: 200-6.
  14. Benhamou JP. Cholangite sclérosante primitive. Rev Prat 2000;50:2146-9.
  15. Majoie C, Smits N, Phoa S, Reeders J, Jansen P. Primary sclerosing cholangitis : sonographic findings. Abdom Imaging 1995; 20:109-12.
  16. Schrumpf E, Fausa O, Forre O, Dobloug JH, Ritland S, Thorsby E. HLA antigens and immunoregulatory T cells in ulcerative colitis associated with hepatobiliary disease. Scand J Gastroenterol 1982; 17: 187-91.
  17. Fulcher AS, Turner MA, Franklin KJ, et al. Primary sclerosing cholangitis: evaluation with MR cholangiography—a casecontrol study. Radiology 2000 ;215:71-80.
  18. Vitellas KM, Enns RA, Keogan MT, et al. Comparison of MR cholangiopancreatographic techniques with contrast-enhanced cholangiography in the evaluation of sclerosing cholangitis. AJR 2002; 178:327-34.
  19. Chazouillères O, Wendum D. Maladies des voies biliaires intrahépatiques. Diagnostic et principes du traitement. Gastroenterol Clin Biol 2003; 27: 307-18.
  20. Main J, McKenzie H, Yeaman GR et al. Antibody to Saccharomyces cerevisiae (bakers' yeast) in Crohn's disease. BMJ 1988; 297: 1105-6.
  21. Guyader D, Deugnier Y, Brissot P. Cholangite sclérosante primitive. Gastroenterol Clin Biol 1990; 14:850-60.
  22. Loftus EV, Harewood GC, Loftus CG, et al. PSCIBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis. Gut 2005; 54: 91-6.
  23. Saarinen S, Olerup O, Broome U. Increased frequency of autoimmune diseases in patients with primary sclerosing cholangitis. Am J Gastroenterol 2000;95:3195-9.