Case Presentation: A 53-year-old woman with a history of metabolic syndrome and necrotizing pancreatitis presented with new onset ascites. Her story began nine months earlier, when she developed abdominal pain. CT at that time revealed pancreatitis and cholelithiasis without biliary dilation. Lab values were notable for: lipase 55000, total bilirubin (TBIL) 5.3, AST 660, ALT 1806, and alkaline phosphatase (ALP) 159 all of which rapidly normalized. She was admitted to the ICU with septic shock and hypoxic respiratory failure requiring tracheostomy. Unfortunately, she then developed hemorrhagic shock from an arterial bleed into a pancreatic pseudocyst that required 13 units pRBCs. She improved clinically, but six weeks after her hemorrhagic shock her ALP and TBIL increased precipitously to 30x and 6x the normal limit, respectively. MRCP revealed a common bile duct (CBD) cast without ductal dilation while ERCP showed mild intrahepatic strictures. She had a plastic stent placed in her CBD with improvement but not resolution of her LFT abnormalities. She was finally discharged but had multiple readmissions for bacterial cholangitis. When she presented to our team with new onset ascites, exam was pertinent for jaundice, muscle wasting, and tense ascites. Lab values showed: TBIL 13.2 (10.7 direct), ALP 430, and normal transaminases. Repeat MRCP revealed diffuse intrahepatic biliary dilation and strictures. US suggested cirrhosis but biopsy showed stage 2 fibrosis with bile duct proliferation. This admission was complicated by repeat cholangitis necessitating multiple biliary drains that provided marginal improvement. 10 months after initial presentation, a transplant hepatology evaluation was initiated. Unfortunately, she passed away 17 months after first developing pancreatitis due to cerebral edema following profound hyperammonemia.
Discussion: Secondary sclerosing cholangitis (SSC) refers to a group of disorders causing inflammation and stricturing of the biliary tree with insidious onset of cirrhosis and liver failure.1,2 Common causes include longstanding biliary obstruction, ischemia, recurrent infection and pancreatitis, and trauma2. A recently described subtype of SSC occurs in ICU patients without a history of hepatobiliary disease or obvious etiology. This is termed SSC in critically-ill patients (CIP). The etiology of SSC-CIP is unknown but thought to be due to ischemia of bile ducts as the result of mechanical ventilation and vasopressors.1 This patient exhibited multiple classic findings of SSC-CIP including biliary casts, rapid progression to cirrhotic physiology despite lack of biopsy proven cirrhosis, bile duct proliferation on biopsy, and progressive biliary strictures without an obvious etiology following critical illness. Treatment of this entity is early transplant evaluation given the known mortality of 17-40 months.4,5 Unfortunately, our patient was in and out of hospital care for 10 months before transplant evaluation was considered. She passed away 17 months after initial presentation.
Conclusions: SSC is a rare diagnosis with only 35 identified in a 10-year period (1992-2002) among patients at the Mayo Clinic in Rochester.6 SSC-CIP is even rarer but is increasing in prevalence as ICU mortality improves. It requires early transplant evaluation. Hospitalists have a role in recognizing the early subtle signs and symptoms of this diagnosis and advocating for early transplant hepatology evaluation, even without classic radiographic and tissue findings of cirrhosis.