Primary sclerosing cholangitis

ICD-10 K83.0  ·  ICD-11 DB96.2

Primary Sclerosing Cholangitis Presenting with Acute Bacterial Cholangitis

Patients with primary sclerosing cholangitis (PSC) are at elevated risk for acute bacterial cholangitis due to underlying biliary strictures. When acute cholangitis develops in this setting, the diagnostic criteria and management approach are distinct from routine biliary infections.

Clinical Scenario

Acute Bacterial Cholangitis — Diagnostic Criteria

The diagnosis of acute bacterial cholangitis in PSC requires suppurative cholangitis on ERCP, or the following combination:

  • At least one major criterion: body temperature >38 °C, leukocyte count >12/nl, or C-reactive protein >75 mg/L
  • At least two minor criteria: positive bile culture, ALP or total bilirubin rise above 2× ULN, or no other identifiable focus of infection
Management Approach

Patients with severe acute cholangitis in the context of PSC require urgent biliary decompression. Management involves an endoscopic approach targeting the biliary obstruction — full technique, sequencing, and adjunct therapy details are in the complete protocol below.

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References

DOI: 10.1016/j.jhep.2022.05.011

According to this definition the diagnosis of acute bacterial cholangitis requires either a single criterion (suppurative cholangitis on ERCP), or at least 1 major criterion (body temperature >38 °C, leukocyte count >12/nl or C-reactive protein >75 mg/L), and at least 2 minor criteria (positive bile culture, increase in ALP or total bilirubin above 2x ULN, no other focus of infection).

Patients with severe acute cholangitis and high-grade bile duct strictures are at high risk of mortality and require urgent biliary decompression.

Most patients will respond to endoscopic drainage of the obstruction in combination with antibiotics.

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