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2014 crohns colitis assoc concord hospital forum psc rupert leong

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Primary Sclerosing Cholangitis

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2014 crohns colitis assoc concord hospital forum psc rupert leong

  1. 1. Rupert WL LeongRupert WL Leong MBBS, MD, FRACP, AGAFMBBS, MD, FRACP, AGAF Concord Hospital Associate Professor UNSW Bankstown Hospital, Sydney, Australia
  2. 2. What is the bile duct?What is the bile duct? intra-hepatic bile ductintra-hepatic bile duct extra-hepatic bile ductextra-hepatic bile duct Bile: digestion of food; stored in gallbladder Normal PSCPSC
  3. 3. Primary Sclerosing CholangitisPrimary Sclerosing Cholangitis – idiopathic – strictures, beading – fibrosis, inflammation – cholestasis • itchy skin, jaundice • vitamin malabsorption 1 LaRusson NF et al. NEJM 84 – associated with IBD
  4. 4. EpidemiologyEpidemiology • Western: – M 2:1 – 30-40’s – 75% PSC will have IBD (UC > CD) – 4% of IBD will have PSC – point prevalence: 13 per 100,0001 – incidence: 0.91 per 100,0002 1 Kingham J et al. Gastroenterol 04, 2 Bambha K et al. Gastroenterol 03
  5. 5. Associated DiseasesAssociated Diseases • IBD: – UC: milder, more extensive, rectal sparing, CRC risk1 – rare: Crohn’s colitis, coeliac, sarcoid, thyroiditis • genetic, molecular: – HLA B8, DR32 ; DRw52a3 , ICAM-14 – MMP5 1 Florin Gut 04, 2 Donaldson Hepatol 91, 3 Prochazka NEJM 92, 4 Yang J Hepatol 04, 5 Satsangi Gastroenterol 01
  6. 6. DiagnosisDiagnosis • incidental abnormal LFT Symptom or Sign Frequency (%) Fatigue 7575 Pruritus 7070 Jaundice, cholestasis 6565 Weight loss, anorexia 40 Fever 25 Hepatomegaly 55 Splenomegaly 30 Hyperpigmentation 25 Xanthomas 4
  7. 7. DiagnosisDiagnosis • cholangiography: beading, strictures, pruning – 1 MRCP – ERCP: • “stent” dominant stricture • 18% complication rate2 – CT cholangiogram 1 Weber C et al. Endosc 08 ; 2 Enns R et al. Can J Gastroenterol 03
  8. 8. PSC
  9. 9. DiagnosisDiagnosis • liver biopsy: non-specific
  10. 10. Ursodeoxycholic AcidUrsodeoxycholic Acid …fever and inflammation, improve eyesight, keep the liver healthy and break down gallstones.
  11. 11. Ursodeoxycholic AcidUrsodeoxycholic Acid • primary biliary cirrhosis • pruritus • hepatitis • prevent bowel cancer • dissolves gallstones • “harmless” (rarely diarrhoea)
  12. 12. Ursodeoxycholic AcidUrsodeoxycholic Acid • synthetic – lithocholic acid – 7b-hydroxylation – Fusarium equiseti
  13. 13. UDCA: low – medium doseUDCA: low – medium dose No Dose Biochemical Histological Mayo score response response or survival Author N = Dose Biochem response Histo response Mayo or survival Van Thiel 92 48 600mg/d (18/12) Yes N/A N/A Stiehl 94 20 750mg/d (1yr) Yes Yes N/A De Maria 96 59 600mg/d (2yr) No No N/A Lindor 97 105 13-15mg/kg/d (2yr) Yes No No van Hoogsraten 98 48 10mg/kg/d (2yr) Yes Trend No Mitchell 01 26 20mg/kg/d (2yr) Yes Yes N/A Harnois 01 23 25-30mg/kg/d (2yr) Yes Yes Yes 3 Olsson 05 219 15-23mg/kg/d (5yr) Yes N/A Trend 4 Cullen 08 31 30mg/kg/d (2yr) Yes N/A Yes • PSC: 8 – 15mg/kg/d
  14. 14. UDCA: high doseUDCA: high dose No Dose Biochemical Histological Mayo score response response or survival Author N = Dose Biochem response Histo response Mayo or survival Van Thiel 92 48 600mg/d (18/12) Yes N/A N/A Stiehl 94 20 750mg/d (1yr) Yes Yes N/A De Maria 96 59 600mg/d (2yr) No No N/A Lindor 97 105 13-15mg/kg/d (2yr) Yes No No van Hoogsraten 98 48 10mg/kg/d (2yr) Yes Trend No 1 Mitchell 01 26 20mg/kg/d (2yr) Yes Yes N/A 2 Harnois 01 23 25-30mg/kg/d (2yr) Yes Yes Yes 3 Olsson 05 219 15-23mg/kg/d (5yr) Yes N/A Trend 4 Cullen 08 31 30mg/kg/d (2yr) Yes N/A Yes • 20 – 30mg/kg/d 1 Mitchell Gastroenterol 01; 2 Harnois Am J Gastroenterol 01; 3 Olsson Gastroenterol 05; 4 Cullen. J Hepatol 08
  15. 15. UDCAUDCA • high dose: 28-30mg/kg/d 1 Lindor et al. Hepatol 10 placebo n = 74placebo n = 74 UDCA n =76UDCA n =76 cirrhosis, varices, cholangiocarcinoma, liver transplantation, death terminated at 6 years n=15n=15 n=22n=22 high dose UDCA 2.3 x greater risk 1° endpoint (P < 0.01) 2.1 x death, transplantation, minimal listing criteria (P = 0.038)
  16. 16. TreatmentTreatment • ineffective – steroids, budesonide – colchicine, penicillamine, nicotine – immunomodulators – infliximab, etanercept – minocycline1 , UDCA combinations2 – vedolizumab 1 Silveira M et al. Am J Gastroenterol 09; 2 Schramm C et al. Ann Int Med 99
  17. 17. ComplicationsComplications PRIMARY SCLEROSING CHOLANGITIS CIRRHOSIS Liver Failure Portal Hypertension MALIGNANCY Colorectal (UC) Cholangio- carcinoma Pancreatic gallbladder CHOLESTASIS Osteoporosis Fat soluble vitamin Cholangitis Stricture Pruritus
  18. 18. Liver TransplantationLiver Transplantation • indications: – intractable pruritus, fatigue – recurrent refractory cholangitis – cirrhosis – Mayo Clinic Score > 5 • R = (0.0295 * (age in years)) + (0.5373 * LN(total bilirubin in mg/dL)) - (0.8389 * (serum albumin in g/dL)) + (0.5380 * LN(AST in IU/L) + (1.2426 * (points for variceal bleeding)) – exclude cholangiocarcinoma
  19. 19. Local Data:Local Data: Rosy Wang, Ken Liu, Crispin CorteRosy Wang, Ken Liu, Crispin Corte • 206 PSC patients – 61% males – median age 41 years – median follow up 19 years (3,868 patient- years) – 77% with IBD; 23% PSC-alone
  20. 20. ConclusionsConclusions • PSC: small proportion of IBD patients – increased cancer risks • hepatobiliary • colorectal – intensive surveillance – local data to provide new recommendations
  21. 21. IBD Concord / BankstownIBD Concord / Bankstown • 10 years – first IBD clinics – first IBD nurses – continuous clinical drug trials – first mesenchymal stem cells study – first IBD pharmacists – clinical excellence in research
  22. 22. A.McAvoy 22 Australian CrohnAustralian Crohn ’’s &s & Colitis AssociationColitis Association (ACCA)(ACCA) IBD Clinical Trials Coordinator Angela McAvoy AM
  23. 23. IBD Concord / BankstownIBD Concord / Bankstown • new initiatives: – “IBD Sydney” – forum for consultant gastroenterologists • rural connection via Skype – support regulatory approval IBD drugs • vedolizumab • infliximab for UC – “IBD Consensus Working Group” – acute severe ulcerative colitis
  24. 24. Australian Bowel FoundationAustralian Bowel Foundation • new gastrointestinal foundation – funding IBD nurses – seeding grants for research – charity, tax deductable donations – fellowship funding – investment – education – focus on PSC – registry • disbursement biomarker patent
  25. 25. • need: – executives – professional associates – ‘professional’ charity organisers • if interested: rupertleong@hotmail.com

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