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Secondary Sclerosing Cholangitis

2NDRY Sclerosing Cholangitis


Presentation

  • Still rarer is its presentation as sclerosing cholangitis. A 50 year male presented with acute onset jaundice, significant weight loss and elevated liver enzymes with clinico-radiological suspicion of cholangiocarcinoma.[ncbi.nlm.nih.gov]
  • The clinical presentation and outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were reviewed.Cholestasis was the first sign of SSC-CIP. GGT was the predominant enzyme of cholestasis.[ncbi.nlm.nih.gov]
  • Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection.[orpha.net]
  • Case presentation A 48-year-old woman presented with an episode of acute biliary pancreatitis that was complicated by pancreatic abcess formation. After 3 months she had an episode of severe pyogenic (E.[doi.org]
  • Here we present a rare case of SSC due to gallbladder adenocarcinoma.[karger.com]
Turkish
  • Primary sclerosing cholangitis in Turkish patients. Characteristic features and prognosis. Hepatobil Pancreat Dis Int. 2009; 8 : 312-5[hepatmon.com]
  • Turkish A, Luo JJ, Lefkowitch JH. Ketamine abuse, biliary tract disease, and secondary sclerosing cholangitis. Hepatol Baltim Md. 2013 Aug;58(2):825–7. CrossRef Google Scholar 10. El Hajj II, Malik SM, Alwakeel HR, Shaikh OS, Sasatomi E, Kandil HM.[link.springer.com]
Regurgitation
  • Regurgitation of bile acids from leaky bile ducts causes sclerosing cholangitis in Mdr2 ( Abcb4 ) knockout mice. Gastroenterology 127, 261–274 (2004). Download references Acknowledgements Charles P.[nature.com]
Jaundice
  • Overall 70% of patients with sclerosing cholangitis had jaundice vs. 25% without sclerosing cholangitis (p CONCLUSIONS: Our results indicate that drugs can lead to bile duct injury visualized on imaging.[ncbi.nlm.nih.gov]
  • A 50 year male presented with acute onset jaundice, significant weight loss and elevated liver enzymes with clinico-radiological suspicion of cholangiocarcinoma.[ncbi.nlm.nih.gov]
  • Later presentation includes abdominal pain, jaundice, pruritus, fever and bacterial cholangitis from ascending infection.[orpha.net]
  • A 25 years old lady, 34 weeks primigravida was referred from the Emergency Department to the Medical Unit Khyber Teaching Hospital-MTI, Peshawar with four weeks of fever, progressive jaundice, pruritus, night sweats and weight loss.[ncbi.nlm.nih.gov]
  • Cholestatic jaundice as a paraneoplastic feature of secondary sclerosing cholangitis is rare in Hodgkin’s lymphoma.[jamc.ayubmed.edu.pk]
Hepatomegaly
  • Hepatomegaly (may also have splenomegaly at presentation). Later stages: signs of cirrhosis, portal hypertension or hepatic failure.[patient.info]
  • ERCP) Hepatomegaly / splenomegaly – approx 50% of patients Investigations P-ANCA – a serum antibody is found in 60% of cases LFT’s: ALP, bilirubin MRCP / ERCP – are able to visualise bile ductal abnormalities.[almostadoctor.co.uk]
  • Additional symptoms include a general feeling of ill health (malaise); abdominal pain, especially the upper right portion of the abdomen; nausea; dark urine; light-colored stools; unintended weight loss, and/or abnormal enlargement of the liver (hepatomegaly[rarediseases.org]
  • Hepatomegaly and splenomegaly are the most frequent findings at clinical examination at the time of diagnosis in PSC. Osteopenic bone disease is a complication of advanced PSC, although less frequent than that reported in PBC.[easl.eu]
  • Drugs that cause cholestasis with bile duct injury often are accompanied by additional clinical features, such as fever, rigors, jaundice, and tender hepatomegaly mimicking acute cholangitis.[clevelandclinicmeded.com]
Scleral Icterus
  • A 55-year-old morbidly obese male presented electively with a 2-week history of low back pain and scleral icterus for 2 days. He also described severe epigastric pain that worsened postprandially and a 13 kg weight loss over the previous month.[karger.com]
Scleral Icterus
  • A 55-year-old morbidly obese male presented electively with a 2-week history of low back pain and scleral icterus for 2 days. He also described severe epigastric pain that worsened postprandially and a 13 kg weight loss over the previous month.[karger.com]
Low Back Pain
  • Case Report A 55-year-old morbidly obese male, with an underlying history of hypertension and dyslipidemia, presented electively, on an outpatient basis, with a 2-week history of low back pain.[karger.com]
Back Pain
  • Case Report A 55-year-old morbidly obese male, with an underlying history of hypertension and dyslipidemia, presented electively, on an outpatient basis, with a 2-week history of low back pain.[karger.com]

Workup

  • Taylor and Jinxing Yu, MRI in the workup of biliary tract filling defects, Journal of Magnetic Resonance Imaging, 37, 5, (1020-1034), (2013). Christoph Jüngst, Thomas Berg, Jun Cheng, Richard M. Green, Jidong Jia, Andrew L.[doi.org]
  • Patients presenting with elevated liver enzymes require a full diagnostic workup and the differential diagnosis of potential hepatopathies is wide.[journals.plos.org]

Treatment

  • BACKGROUND AND STUDY AIMS: We present ten patients who developed secondary sclerosing cholangitis following long-term treatment in an intensive care unit (ICU) between 1999 and 2004.[ncbi.nlm.nih.gov]
  • Our aim was to analyze the microbial spectrum in bile of patients with SSC-CIP and to evaluate the potential impact on the empiric antibiotic treatment in these patients.[ncbi.nlm.nih.gov]
  • All patients who developed secondary sclerosing cholangitis in association with intensive care treatment were included. Possible trigger factors during the course of the initial intensive care treatment were recorded.[ncbi.nlm.nih.gov]
  • A new variant of SSC occurring after long-term treatment in intensive care units (ICU) has been recently described and characterized from the clinical point of view.[ncbi.nlm.nih.gov]
  • These abnormalities were considered not to be amenable for endoscopic or percutaneous transhepatic treatment. Antibiotic treatment was continued and cholangitis did not recur during 14 months of follow-up.[doi.org]

Prognosis

  • SSC-CIP was associated with worse prognosis; transplant-free survival was 40 months (mean).Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other[ncbi.nlm.nih.gov]
  • Secondary sclerosing cholangitis due to drugs or autoimmune causes seem to have the best prognosis. However, causes such as critical illness seem to have worse outcomes.[ncbi.nlm.nih.gov]
  • Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is a destructive cholangiopathy with a poor prognosis.[ncbi.nlm.nih.gov]
  • Characteristic features and prognosis. Hepatobil Pancreat Dis Int. 2009; 8 : 312-5[hepatmon.com]
  • Increased awareness and early detection of the disease and its complications is considered to be crucial to improve the poor prognosis.[dovepress.com]

Etiology

  • He was diagnosed as definite eosinophilic cholecystitis with sclerosing cholangitis with unknown etiology. The possible etiology of sclerosing cholangitis was consequent fibrosis from previous eosinophilic infiltration in the bile duct.[ncbi.nlm.nih.gov]
  • Yet, because of the low incidence and varying etiologies of this disease, the literature is not robust. There are mainly case reports and small studies evaluating the disease.[ncbi.nlm.nih.gov]
  • Liver biopsy, performed to exclude other etiologies of liver failure, demonstrated biliary cholestasis.[karger.com]
  • The diagnosis was confirmed by chart review and information about age, gender, etiology, therapy, and clinical course was sought.[ncbi.nlm.nih.gov]
  • Etiology infectious chronic cholangitis biliary cryptosporidiosis (immunodeficiencies) Langerhans cell histiocytosis choledocholithiasis sickle cell disease total parenteral nutrition Hodgkin disease primary intestinal T-cell lymphoma ( 9893108 ) cystic[humpath.com]

Epidemiology

  • The volume focuses on new data about risk factors for PSC, its natural history of the disease, and the epidemiology and genetics of PSC, while providing a comprehensive overview on current and future therapies for PSC, management of cholangiocarcinoma[books.google.com]
  • Epidemiology 75% of cases are associated with IBD (usually ulcerative collitis ).[almostadoctor.co.uk]
  • Secondary sclerosing cholangitis includes conditions where infections, thrombosis, iatrogenic causes or trauma can give rise to similar clinical characteristics to PSC. [ 1 ] Epidemiology and associated diseases [ 1, 2 ] PSC is a rare disease with a prevalence[patient.info]
  • Ponsioen, Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: A systematic review, Journal of Hepatology, 56, 5, (1181), (2012). Efthymia Alexopoulou, Polis E. Xenophontos, Nikos Economopoulos, Themistoklis N.[doi.org]
  • METHODS: The Surveillance, Epidemiology, and End Results (SEER) database from 1988 to 2009 was used to obtain data on patients with thyroid cancer.[epiprev.it]
Sex distribution
Age distribution

Pathophysiology

  • CONCLUSIONS: The development of secondary sclerosing cholangitis in patients who have received long-term treatment in an ICU is a rare event of unknown pathophysiology, but patients demonstrate characteristic findings on ERCP.[ncbi.nlm.nih.gov]
  • Sclerosing cholangitis following severe trauma: description of a remarkable disease entity with emphasis on possible pathophysiologic mechanisms. World J. Gastroenterol. 11, 4199–4205 (2005). 46. Jaeger, C. et al.[nature.com]
  • Approximately 80% of patients with PSC have inflammatory bowel disease, more commonly ulcerative colitis than Crohn’s disease. 21 Pathophysiology and Natural History The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune[clevelandclinicmeded.com]
  • J Hepatobiliary Pancreat Surg 14 35-45 2007 [Refereed] Definitions, pathophysiology, and epidemiology of acute cholangitis and cholecystitis: Tokyo Guidelines.[researchmap.jp]

Prevention

  • Antibiotic treatment and drainage led to rapid improvement on each occasion, but did not prevent new episodes.[doi.org]
  • The aim of the study is to assess the efficacy of rituximab to prevent recurrence of PSC.[upload.umin.ac.jp]
  • Prevention It is not possible to prevent PSC because the cause remains unknown. Liver damage and cirrhosis is often presumed to be caused by drinking too much alcohol, however PSC is not related to alcohol in any way.[britishlivertrust.org.uk]
  • UDCA – a hydrophilic bile acid, used to prevent liver damage by reducing the number of cholestatic liver enzymes. This alters the metabolism of cholesterol and micelles, and makes them break down more readily.[almostadoctor.co.uk]
  • This is due to the fact that health care in Japan offers annual check-ups, as part of their prevention program. This allows relatively early detection of diseases, in this case CCA.[pscpatientseurope.org]

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