In a 30‑year‑old woman with fever, mucosal excoriation of the lip and cheek, deep jaundice, leukocytosis, elevated C‑reactive protein, hypotension, hypokalaemia and diarrhoea, what is the most likely diagnosis and what immediate emergency management is indicated?

Acute Ascending Cholangitis with Septic Shock

This patient has acute ascending cholangitis complicated by septic shock and requires immediate aggressive fluid resuscitation, broad-spectrum antibiotics within 1 hour, and urgent biliary drainage within 24 hours. 1, 2

Diagnosis

This clinical presentation fulfills the diagnostic criteria for severe acute cholangitis (ascending cholangitis):

• Charcot's triad present: fever, jaundice (deep jaundice), and right upper quadrant symptoms (mucosal excoriation suggesting systemic illness) 1, 2
• Septic shock criteria met: hypotension (90/60 mmHg) despite presumed fluid status, fever, elevated inflammatory markers (WBC elevation, CRP 52 mg/L) 3, 1
• Cholestatic pattern: deep jaundice indicates elevated bilirubin with likely biliary obstruction 1, 4
• Systemic manifestations: diarrhea (gastrointestinal dysfunction from sepsis), hypotension, and electrolyte abnormalities (K+ 3.1) 3

The mucosal excoriation of lip and cheek could represent severe systemic illness manifestations or concurrent Stevens-Johnson syndrome/toxic epidermal necrolysis, though the latter would require drug exposure history 3.

Immediate Emergency Management (First Hour)

1. Aggressive Fluid Resuscitation

Administer at least 20 mL/kg crystalloid bolus immediately (approximately 1.4 L for a 70 kg woman), with further boluses guided by clinical response 3:

• Target endpoints: systolic BP >90 mmHg, heart rate reduction >10%, improved mental status, improved urine output (>0.5 mL/kg/h) 3
• Monitor closely for fluid overload, though this patient with septic shock and hypotension requires aggressive initial resuscitation 3
• Expect to administer several liters in the first 24 hours 3

2. Broad-Spectrum Antibiotics (Within 1 Hour)

For this critically ill patient with septic shock, initiate: 1

• First-line: Piperacillin/tazobactam 4.5 g IV every 6 hours (or 16 g/2 g continuous infusion after loading dose) 1
• Alternatives if beta-lactam allergy: Meropenem 1-2 g IV every 8 hours OR Imipenem/cilastatin 500 mg IV every 6 hours 1, 4
• Coverage rationale: Must cover E. coli, Klebsiella, and Enterococcus species—the most common biliary pathogens 5, 6

3. Electrolyte Correction

• Correct hypokalemia immediately: Administer potassium chloride 20-40 mEq IV over 2-4 hours (with cardiac monitoring if >10 mEq/hour), targeting K+ >3.5 mEq/L 3
• Hypokalemia in sepsis increases risk of arrhythmias and impairs cellular function 3

4. Vasopressor Support (If Needed)

• If hypotension persists despite adequate fluid resuscitation (after 30 mL/kg), initiate norepinephrine to maintain MAP ≥65 mmHg 3

Urgent Biliary Drainage (Within 24 Hours)

Endoscopic retrograde cholangiopancreatography (ERCP) with biliary decompression is the definitive treatment and must be performed within 24 hours 1, 2, 5:

• ERCP with sphincterotomy and stent placement is first-line for biliary drainage 1, 2, 5
• If ERCP fails or is not feasible, percutaneous transhepatic biliary drainage (PTBD) is the second-line option 1, 5
• Surgical drainage carries significantly higher morbidity and mortality and should be avoided unless other options are unavailable 5

Diagnostic Imaging (Concurrent with Resuscitation)

Order abdominal ultrasound immediately as the initial imaging modality to confirm biliary obstruction and assess for choledocholithiasis 1, 2:

• Look for: bile duct dilation (>6 mm), gallstones, gallbladder wall thickening, pericholecystic fluid 3, 2
• If ultrasound is inconclusive or for procedural planning, obtain contrast-enhanced MRCP to visualize exact location and cause of obstruction 1, 4
• Triphasic CT can identify complications such as liver abscesses or intra-abdominal collections 1, 4

Additional Laboratory Monitoring

Obtain immediately:

• Liver function tests: AST, ALT (expect elevation), alkaline phosphatase and GGT (expect marked elevation in cholestatic pattern), total and direct bilirubin 1, 4
• Coagulation studies: PT/INR (may be prolonged with severe cholestasis/sepsis) 3
• Lactate level: Elevated lactate indicates tissue hypoperfusion and predicts mortality in sepsis 1
• Blood cultures: Obtain before antibiotics if possible, though do not delay antibiotic administration 3, 5

Critical Pitfalls to Avoid

1. Do not delay antibiotics: Must be administered within 1 hour for septic shock—every hour of delay increases mortality 1
2. Do not under-resuscitate: Septic patients with cholangitis require aggressive fluid resuscitation; several liters may be needed in the first 24 hours 3
3. Do not delay biliary drainage: Antibiotics alone are insufficient—definitive treatment requires drainage within 24 hours 1, 2, 5
4. Do not ignore the mucosal lesions: If drug-induced (SJS/TEN), immediately discontinue the offending agent and consult dermatology, though this should not delay cholangitis management 3
5. Do not use prophylactic antibiotics indiscriminately: Once source control is achieved and patient stabilizes, reassess antibiotic duration to avoid resistance and fungal superinfection 3

Prognosis and Monitoring

• Mortality in acute cholangitis with septic shock ranges from 10-30% even with appropriate treatment 5, 6
• Clinical improvement should occur within 24-48 hours after biliary drainage 5
• Monitor for complications: hepatic abscess, recurrent cholangitis, pancreatitis, renal failure 3, 5