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May 20, 2026American Journal of Respiratory and Critical Care Medicine0 citations

A102-13 A Case of Rapid Onset Secondary Sclerosing Cholangitis Following Veno-venous Extracorporeal Membrane Oxygenation (ECMO)

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TCT CatonTemple University HospitalKAK AbbasTemple University HospitalJKJ KanaparthiTemple University Hospital

Key Points

  • This case aims to illustrate the rapid onset of secondary sclerosing cholangitis in a patient following veno-venous ECMO.
  • Reported a case of a 72-year-old woman with critical illness post-lung transplantation treated with ECMO.
  • Monitored liver function tests and conducted diagnostic imaging to assess liver condition during ECMO.
  • Initiated treatment with ursodiol amidst deterioration of liver function.
  • Liver function tests showed significant increases in alkaline phosphatase (peak 1,318 U/L) and total bilirubin (24.2 mg/dL) after ECMO initiation.
  • Patient presented with cholestatic liver dysfunction and unremarkable viral hepatitis studies.
  • Despite treatment, the patient's condition deteriorated, leading to death.

Abstract

Abstract Extracorporeal membrane oxygenation (ECMO) is an established life-saving intervention for patients with refractory cardiopulmonary failure. Recent literature identifies a rare form of secondary sclerosing cholangitis in critically ill patients (SSC-CIP), although few reports of these cases following ECMO exist 1. A 72-year-old woman with a history of scleroderma, interstitial lung disease, and ductal breast cancer status post resection and radiation underwent left lung transplantation. Induction was with basiliximab; post-operative immunosuppressant regimen consisting of tacrolimus, mycophenolate mofetil, and methylprednisolone. The post-operative course was complicated by pericardial effusion with tamponade physiology requiring pericardiectomy and a duodenal perforation managed conservatively. The patient experienced a prolonged hospital course with ventilator-dependent respiratory failure following acute respiratory distress syndrome due to pneumonia. Despite proning and treatment with steroids and antibiotics, her hypoxemia worsened, necessitating veno-venous ECMO. Liver function tests (LFTs) were within normal limits prior to cannulation. After approximately eight days, LFTs began to rise, demonstrating a cholestatic pattern, with alkaline phosphatase (ALP) peaking at 1,318 U/L and total bilirubin at 24.2 mg/dL (Table 1). Viral hepatitis studies and comprehensive chronic liver disease work-up were unremarkable except for known positive Antinuclear Antibody (ANA), elevated immunoglobulin G (IgG), and anti-smooth muscle antibody (ASMA) at 1:160. Neither abdominal ultrasound nor computed tomography of the abdomen and pelvis with intravenous contrast showed biliary obstruction, masses, or intra-abdominal abscesses (Figure 1). Ursodiol was initiated, resulting in modest improvement in ALP to the 500s. Magnetic resonance cholangiopancreatography (MRCP) and liver biopsy were unable to be performed due to the patient’s critical condition. The patient’s clinical status continued to deteriorate, and she ultimately died. This case illustrates a rare presentation of presumed secondary sclerosing cholangitis (SSC) associated with ECMO. SSC is characterized by rapid progression to biliary fibrosis and cirrhosis. The condition typically arises in the context of known injuries or prolonged biliary obstruction. However, SSC has been reported to occur rarely among critically ill patients, likely secondary to ischemic injury. Cholestatic liver dysfunction develops, initially marked by elevated gamma-glutamyl transpeptidase followed by significant increases in ALP with a moderate rise in bilirubin. MRCP is useful for identifying multifocal bile duct strictures and beading, which are characteristics of SSC-CIP 2. Early identification may facilitate interventions such as ursodeoxycholic acid, endoscopic retrograde cholangiopancreatography, or liver transplantation, although prognosis remains poor. As ECMO use increases, elucidating the relationship between SSC-CIP and ECMO as a potential causative factor is paramount. This abstract is funded by: None

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Cite This Study

Caton et al. (2026) studied this question.

synapsesocial.com/papers/6a0d5114f03e14405aa9d623https://doi.org/10.1093/ajrccm/aamag162.4660
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