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cholangitis

- inflammation of the bile duct or entire biliary tree - also see ascending cholangitis Etiology: - biliary obstruction & stasis, gallstones Pathology: 1) biliary obstruction & stasis 2) hepatitis 3) bacterial infection typically ascending from duodenum (ascending cholangitis) 4) shock Clinical manifestations: 1) right upper quadrant abdominal pain, colicky in nature* 2) fever/chills* 3) jaundice* 3) nausea/vomiting 5) tender, enlarged liver 6) palpable spleen (occasionally) 7) gallstone pancreatitis * Charcot's triad [2] Diagnostic criteria: - cholestatic pattern of liver enzyme elevation - mainly alkaline phosphatase in serum - presence of antimitochondrial antibody in serum or sp100 Ab or gp210 Ab - histologic findings consistent with primary biliary cholangitis Laboratory: 1) complete blood count: leukocytosis 2) liver function tests a) serum alkaline phosphatase: may be very high - elevated serum gamma-glutamyl transferase (serum GGT) b) serum bilirubin (conjugated): > 4 mg/dL [2] c) serum transaminases: may be > 1000 U/L [2] 3) elevated serum amylase 4) urinalysis: elevated urine bilirubin 5) mitochondrial antibody in serum [6] 6) antinuclear antibody in serum, rim pattern 6) consider genetic tests for inherited cholestatic syndromes Special laboratory: - endoscopic retrograde cholangiopancreatography (ERCP) - next procedure after ultrasound [2] - provides decompression with stone extraction or stent placement for biliary stricture [2] - gold standard for diagnosis of primary sclerosing cholangitis [2] - endoscopic ultrasound only if indicated after ERCP - liver biopsy - negative serology & MRI imaging for unexplained intrahepatic cholestasis [6] - serum ALT > 5X upper limit of normal [9] Radiology: - ultrasound of gall bladder & biliary tree first line - magnetic resonance imaging - magnetic resonance cholangiopancreatography for unexplained cholestasis [6] - bone density scan (DEXA) to assess risk for osteoporosis [6] Complications: - primary sclerosing cholangitis is associated with an increased risk for cholangiocarcinoma [2] - cirrhosis - shock & mental status changes, potentially life-threatening [2] Management: 1) broad-spectrum intravenous antibiotics a) target anaerobes, gram-negative Enterobacteriaceae, multi-drug resistant gram-negative bacilli, Enterococcus, & MRSA b) use unit- or hospital-specific resistance patterns as guide c) combination of 1] metronidazole plus 2] imipenem-cilastatin, meropenem, doripenem, piperacillin-tazobactam, ciprofloxacin, levofloxacin, or cefepime, (beta-lactam/beta-lactamase inhibitor or 3rd generation cephalosporin) [2] plus 3] vancomycin (not included in MKSAP19 recommendations [2] d) alternative regimen 1] aztreonam plus 2] metronidazole, plus 3] vancomycin e) 4 days of post-drainage antibiotics with similar outcomes as 8 days [11] 2) endoscopic retrograde cholangiopancreatography (ERCP) to remove bile duct stone(s) & establish drainage (sphincterotomy) if improvement ubsatisfactory with antibiotic therapy [2] - in-hospital mortality lower with emergent than urgent ERCP [10] 3) pregnancy generally well-tolerated in non-cirrhotic patients [6] 4) avoid surgery; associated with increased mortality [2] 5) poor prognostic indicators - early age of diagnosis (< 45 years) & advanced disease at presentation [6] 6) ursodeoxycholic acid 13-15 mg/kg/day for life [6] 7) consider adjunctive therapy with obeticholic acid [6] 8) addition of bezafibrate to ursodeoxycholic acid may result in a complete biochemical response [8] 9) immunosuppressive treatment in addition to ursodeoxycholic acid may be necessary in patients with autoimmune hepatitis [6] 10) cholestyramine 1st-line therapy for pruritus [6] - rifampicin (150-300 mg/day) 2nd-line for pruritus 11) vitamin D & calcium supplement [6] - bisphosphonate safe & effective for osteoporosis 12) liver transplantation for cirrhosis [6] - ursodeoxycholic acid safe & effective after liver transplantation

Related

biliary colic (biliary spasm) cholecystitis cholelithiasis (gallstones)

Specific

ascending cholangitis (cholangitis lenta) cholangiolitis HIV-associated cholangitis Mirrizi's syndrome primary sclerosing cholangitis (PSC) recurrent pyogenic cholangitis vanishing bile duct syndrome (idiopathic ductopenia)

General

cholangiopathy hepatobiliary infection

References

  1. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 873
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Prescriber's Letter 17(3): 2010 CHART: Antibiotics for Complicated Intra-Abdominal Infections GUIDELINES: Diagnosis and Management of Complicated Intra- abdominal Infections Detail-Document#: 260321 (subscription needed) http://www.prescribersletter.com
  4. Lee JG. Diagnosis and management of acute cholangitis. Nat Rev Gastroenterol Hepatol. 2009 Sep;6(9):533-41. PMID: 19652653
  5. Khashab MA, Tariq A, Tariq U et al Delayed and unsuccessful endoscopic retrograde cholangiopancreatography are associated with worse outcomes in patients with acute cholangitis. Clin Gastroenterol Hepatol. 2012 Oct;10(10):1157-61. PMID: 22507875
  6. Anello J, Feinberg B, Heinegg J et al Primary Biliary Cholangitis Guidelines on primary biliary cholangitis by the European Association for the Study of the Liver. Medscape: New Guidelines and Recommendations, August 2017. http://reference.medscape.com/viewarticle/884517
  7. Hirschfield GM, Dyson JK, Alexander GJM, et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut. 2018 Mar 28. PMID: 29593060 Free full text http://gut.bmj.com/content/early/2018/03/28/gutjnl-2017-31525
  8. Corpechot C, Chazouilleres O, Rousseau A et al A Placebo-Controlled Trial of Bezafibrate in Primary Biliary Cholangitis. N Engl J Med 2018; 378:2171-2181. June 7, 2018 PMID: 29874528 https://www.nejm.org/doi/full/10.1056/NEJMoa1714519 - Carey EJ Progress in Primary Biliary Cholangitis. N Engl J Med 2018; 378:2234-2235. June 7, 2018 PMID: 29874531 https://www.nejm.org/doi/full/10.1056/NEJMe1804945
  9. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology 2018 Aug 2 PMID: 30070375 https://aasldpubs.onlinelibrary.wiley.com/doi/abs/10.1002/hep.30145
  10. Iqbal U, Khara H, Hu Y et al. Emergent versus urgent ERCP in acute cholangitis: A systematic review and meta-analysis. Gastrointest Endosc 2019 Oct 16. PMID: 31628955 https://www.giejournal.org/article/S0016-5107(19)32318-1/pdf
  11. Srinu D et al. Conventional vs short duration of antibiotics in patients with moderate or severe cholangitis: Noninferiority randomized trial. Am J Gastroenterol 2023 Oct 9; [e-pub] PMID: 37732816 https://journals.lww.com/ajg/abstract/9900/conventional_vs_short_duration_of_antibiotics_in.877.aspx