Cholangitis: A Comprehensive Medical Overview

Introduction

Cholangitis represents a significant clinical entity within hepatobiliary medicine, posing a considerable burden on healthcare systems worldwide. It refers to inflammation of the biliary tree, typically resulting from infection or autoimmune mechanisms, and is associated with a spectrum of acute and chronic presentations.

The condition, if not promptly recognised and managed, can result in severe morbidity and mortality due to rapid progression to sepsis and multi-organ dysfunction. The epidemiology of cholangitis varies globally, with incidence rates influenced by geographical differences in the prevalence of gallstone disease, biliary tract malignancies, and underlying immunological disorders.

Significance and Epidemiology

Cholangitis is a potentially life-threatening disease that requires prompt diagnosis and intervention. The acute form, known as acute cholangitis or ascending cholangitis, is a medical emergency commonly encountered in gastroenterology and surgical practice. Chronic forms, including primary sclerosing cholangitis (PSC), carry long-term consequences, including the risk of cholangiocarcinoma and progressive liver failure.

Definition and Classification

Cholangitis is defined as inflammation of the biliary tract, most commonly due to bacterial infection secondary to biliary obstruction. The disease may be classified based on clinical course (acute vs. chronic), aetiology (infectious vs. autoimmune), and pathological features.

Acute vs. Chronic Cholangitis

• Acute Cholangitis (Ascending Cholangitis):
• An acute, potentially life-threatening infection of the biliary tree, usually associated with obstruction and bacterial invasion. It is characterised by rapid onset and progression.
• Chronic Cholangitis:
• Refers to long-standing inflammation of the bile ducts, often resulting from autoimmune or idiopathic processes, as seen in primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC).

Primary vs. Secondary Cholangitis

• Primary Cholangitis: Disease arises without an identifiable underlying cause, often immune-mediated (e.g., PSC, PBC).
• Secondary Cholangitis: Results from identifiable causes such as biliary obstruction, infection, or trauma.

Types of Cholangitis

• Ascending (Acute) Cholangitis: Caused by bacterial infection ascending from the duodenum, usually in the setting of biliary obstruction.
• Primary Sclerosing Cholangitis (PSC): Chronic, progressive, idiopathic disease characterised by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts, often associated with inflammatory bowel disease.
• Primary Biliary Cholangitis (PBC): Autoimmune destruction of small intrahepatic bile ducts, formerly known as primary biliary cirrhosis.
• IgG4-related Cholangitis: Immune-mediated disease associated with elevated IgG4 levels and systemic involvement.
• Recurrent Pyogenic Cholangitis: Chronic infection, often associated with intrahepatic stones, more prevalent in Asian populations.

Etiology and Risk Factors

Causes of Cholangitis

Bacterial Infection:

• The most common cause of acute cholangitis, usually due to enteric bacteria such as Escherichia coli, Klebsiella species, Enterococcus, and Enterobacter species. Infection typically occurs when biliary drainage is impaired.

Biliary Obstruction:

• Obstruction of the biliary tract is the principal predisposing factor. Causes include:
• Gallstones (choledocholithiasis)
• Biliary strictures (benign or malignant)
• Neoplasms (cholangiocarcinoma, pancreatic cancer)
• Parasitic infestations (e.g., liver flukes)
• Post-surgical strictures or injury
• Stents or foreign bodies in the biliary tree

Autoimmune Mechanisms:

Responsible for conditions such as PSC and PBC, where immune-mediated destruction or fibrosis of bile ducts occurs.

Parasitic and Fungal Infections:

More common in endemic areas, with organisms such as Clonorchis sinensis and Ascaris lumbricoides.

Viral Infections:

Rarely, viruses such as cytomegalovirus (CMV) or hepatitis viruses can cause cholangitis, especially in immunocompromised patients.

Risk Factors

• Choledocholithiasis (common bile duct stones)
• History of biliary tract surgery or instrumentation (e.g., ERCP, stenting)
• Malignancy of the hepatobiliary or pancreatic region
• Benign biliary strictures (e.g., secondary to chronic pancreatitis)
• Congenital biliary anomalies (e.g., choledochal cysts)
• Immunosuppression (HIV/AIDS, post-transplantation, chemotherapy)
• Inflammatory bowel disease (particularly ulcerative colitis with PSC)
• Presence of biliary stents or drains
• Parasitic infestations (more common in tropical regions)
• Older age
• Male gender (higher risk for PSC)

Pathophysiology

The pathogenesis of cholangitis centres on impaired biliary drainage, leading to infection and inflammation of the bile ducts. The mechanisms differ according to the type of cholangitis but share common pathogenic pathways in acute infection.

Mechanisms of Disease Development

• Biliary Obstruction: Obstruction (by stone, stricture, tumour, etc.) increases intraductal pressure, impairs bile flow, and disrupts the mucosal barrier.
• Bacterial Ascension: Bacteria from the duodenum ascend through the sphincter of Oddi into the biliary tree, particularly when the barrier is compromised.
• Infection and Inflammation: Bacterial colonisation triggers an inflammatory response, leading to neutrophil infiltration, cytokine release, and further tissue damage.
• Systemic Dissemination: Bacteria and toxins may enter the systemic circulation, causing bacteraemia and sepsis.
• Chronic Inflammation and Fibrosis: In chronic cholangitis (e.g., PSC), ongoing inflammation results in progressive fibrosis, strictures, and ultimately biliary cirrhosis.

Immune Response

In autoimmune cholangitis (PSC, PBC), dysregulation of immune tolerance leads to lymphocytic infiltration and destruction of bile duct epithelium. Genetic predisposition, environmental factors, and associated autoimmune conditions contribute to disease initiation and progression.

Clinical Presentation

The clinical features of cholangitis vary according to the type, severity, and underlying aetiology. Acute cholangitis presents with a characteristic set of signs and symptoms, while chronic forms may have a more indolent course.

1.Acute Cholangitis

Charcot’s Triad:

• The classic triad for diagnosis of acute cholangitis, comprising:
• Fever (often with rigors)
• Right upper quadrant abdominal pain
• Jaundice

Reynolds’ Pentad:

• Indicates severe disease with systemic involvement, adding:
• Hypotension (shock)
• Altered mental status (confusion, disorientation)

Other Symptoms:

• Nausea and vomiting
• Malaise and anorexia
• Pruritus (itching)
• Dark urine and pale stools (due to cholestasis)

Physical Examination:

• Fever and tachycardia
• Jaundice (icterus)
• Tenderness in the right upper quadrant
• Signs of sepsis in severe cases

2.Chronic Cholangitis (PSC, PBC)

• Fatigue
• Pruritus
• Progressive jaundice
• Hepatomegaly and splenomegaly
• Signs of chronic liver disease (ascites, spider naevi, palmar erythema)
• Complications such as portal hypertension and hepatic encephalopathy in advanced stages

Diagnosis and Investigations

Early diagnosis of cholangitis is crucial to prevent complications. The approach includes clinical evaluation, laboratory testing, and imaging studies.

Laboratory Tests

• Complete Blood Count (CBC): Leucocytosis with neutrophilia is common in acute cholangitis.
• Liver Function Tests (LFTs): Elevated serum bilirubin (predominantly conjugated), Increased alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT), Mild to moderate elevation of transaminases (AST, ALT)
• Blood Cultures: Essential to detect bacteraemia; positive in up to 50–70% of cases.
• Inflammatory Markers: Elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ESR).
• Autoimmune Markers: Antimitochondrial antibody (AMA) in PBC; perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) in PSC.
• Serum IgG4 levels: May be elevated in IgG4-related cholangitis.

Imaging Studies

• Ultrasonography (USG): First-line imaging for suspected cholangitis; may demonstrate bile duct dilatation, stones, or abscesses.
• Computed Tomography (CT): Useful for detecting complications such as liver abscesses or malignancy.
• Magnetic Resonance Cholangiopancreatography (MRCP): Non-invasive technique to visualise the biliary tree and identify strictures, stones, or masses.
• Endoscopic Retrograde Cholangiopancreatography (ERCP): Gold standard for diagnosis and therapeutic intervention; allows for direct visualisation, sampling, and drainage.
• Endoscopic Ultrasound (EUS): Helpful in detecting small stones or tumours not seen on other imaging.

Diagnostic Criteria

The Tokyo Guidelines (2018) provide standardised criteria for diagnosing acute cholangitis, incorporating clinical, laboratory, and imaging findings:

• Systemic Inflammation: Fever, chills, or laboratory evidence of inflammation (leucocytosis, elevated CRP).
• Cholestasis: Jaundice or abnormal LFTs indicative of bile duct obstruction.
• Imaging: Evidence of biliary dilatation or aetiological factors on imaging.

Definite diagnosis requires at least one item from each category. Suspected cases require two of the three.

Treatment and Management

Management of cholangitis depends on the type, severity, and underlying cause. The primary goals are to control infection, relieve biliary obstruction, and prevent complications. A multidisciplinary approach involving gastroenterologists, interventional radiologists, and surgeons is often required.

Initial Supportive Care

• Hospitalisation: All patients with suspected acute cholangitis should be admitted for monitoring and treatment.
• Fluid Resuscitation: Intravenous fluids to maintain haemodynamic stability and correct dehydration.
• Electrolyte Correction: Monitor and correct electrolyte imbalances.
• Monitoring: Vital signs, urine output, and mental status should be closely observed.

Antibiotic Therapy

Empirical broad-spectrum intravenous antibiotics should be initiated promptly, targeting common enteric pathogens. Antibiotic regimens may include:

• Third-generation cephalosporins (e.g., ceftriaxone) plus metronidazole
• Piperacillin-tazobactam
• Carbapenems (e.g., imipenem) for severe or resistant cases
• Aminoglycosides may be added in critically ill patients

Antibiotic therapy is adjusted based on culture results and clinical response. The duration is typically 7–10 days.

Biliary Drainage

Relief of biliary obstruction is the cornerstone of management in most cases of acute cholangitis. Methods include:

Endoscopic Retrograde Cholangiopancreatography (ERCP):

• Preferred modality for biliary decompression; allows for stone extraction, stent placement, and sphincterotomy.
• Percutaneous Transhepatic Biliary Drainage (PTBD):
• Used when ERCP is not feasible or unsuccessful.

Surgical Intervention:

• Reserved for cases where endoscopic or percutaneous methods fail or are contraindicated.

Definitive Therapy

• Stone Removal: Endoscopic extraction during ERCP or surgical exploration.
• Treatment of Strictures or Tumours: Stenting, dilatation, or surgical resection as appropriate.
• Cholecystectomy: Elective removal of the gallbladder may be indicated after resolution of acute infection to prevent recurrence.

Management of Chronic Cholangitis

• Primary Sclerosing Cholangitis (PSC): No effective medical cure; management focuses on symptom control, surveillance for complications, and liver transplantation in advanced cases.
• Primary Biliary Cholangitis (PBC): Ursodeoxycholic acid (UDCA) is the mainstay; obeticholic acid may be considered in non-responders.
• Immunosuppressive Therapy: Considered in selected autoimmune cases (e.g., corticosteroids, azathioprine for IgG4-related cholangitis).
• Endoscopic Management: For dominant strictures to relieve cholestasis.

Supportive Care

• Analgesia and antipyretics for symptom relief
• Nutritional support, especially in chronic disease
• Management of pruritus (e.g., cholestyramine, rifampicin)
• Vitamin supplementation (fat-soluble vitamins in chronic cholestasis)

Complications

Cholangitis, particularly if untreated or inadequately managed, can result in a range of serious complications:

• Sepsis and Septic Shock: Dissemination of infection can lead to systemic inflammatory response, multi-organ dysfunction, and death.
• Liver Abscess: Localised collection of pus within the liver parenchyma.
• Biliary Cirrhosis: Progressive fibrosis and destruction of bile ducts leading to cirrhosis and portal hypertension.
• Acute Renal Failure: Secondary to sepsis or hypoperfusion.
• Disseminated Intravascular Coagulation (DIC): Life-threatening complication in severe sepsis.
• Recurrent Cholangitis: Especially in the presence of persistent obstruction or stents.
• Cholangiocarcinoma: Increased risk in chronic cholangitis, especially PSC.
• Malnutrition and Fat-Soluble Vitamin Deficiencies: Due to chronic cholestasis.

Prognosis

The prognosis of cholangitis depends on the type, severity, timeliness of intervention, and presence of comorbidities. Acute cholangitis, if treated early with appropriate antibiotics and biliary drainage, has a favourable outcome. Mortality rates have decreased significantly with advances in supportive care and endoscopic techniques, but remain high in severe cases complicated by sepsis or multi-organ failure.

• Factors Affecting Prognosis:
  • Severity of infection (as per Tokyo Guidelines severity grading)
  • Delay in diagnosis or intervention
  • Advanced age and comorbidities (diabetes, malignancy, immunosuppression)
  • Presence of complications (shock, renal failure)

• Mortality Rates: Range from less than 10% in mild cases to over 50% in severe, untreated cases.
• Recurrence: Common in patients with persistent risk factors (e.g., stones, strictures, stents).
• Chronic Cholangitis: PSC carries a poor long-term prognosis, with median survival of 10–12 years from diagnosis in advanced cases. Liver transplantation improves outcomes.

Prevention Strategies

Preventive measures are focused on reducing risk factors, timely management of predisposing conditions, and patient education.

• Timely Treatment of Gallstones: Early cholecystectomy or endoscopic management of choledocholithiasis to prevent obstruction.
• Careful Biliary Instrumentation: Strict aseptic technique during ERCP and other procedures to minimise infection risk.
• Prophylactic Antibiotics: In high-risk patients undergoing biliary interventions.
• Surveillance for High-Risk Groups: Regular monitoring in patients with PSC, PBC, or biliary stents for early detection of complications.
• Control of Parasitic Infections: Deworming programmes in endemic regions.
• Patient Education: Informing patients about the signs and symptoms of cholangitis, importance of medication adherence, and need for follow-up.
• Immunisation: Hepatitis A and B vaccination in patients with chronic liver or biliary disease.
• Lifestyle Measures: Avoiding hepatotoxic substances, maintaining a healthy diet, and managing comorbid conditions.

Nursing Care of Patients with Cholangitis

Nursing Assessment

A thorough nursing assessment forms the foundation for effective care. Components include:

1. Health History

• Onset, duration, and character of abdominal pain
• Presence of fever, chills, or rigors
• History of jaundice, pruritus, or changes in urine/stool color
• Past medical history: gallstones, liver disease, previous biliary interventions
• Medication and allergy history

2.Physical Examination

• Vital signs: monitor for fever, tachycardia, hypotension
• Skin and sclerae: assess for jaundice, pruritus, scratch marks
• Abdomen: inspect for distension, palpate for tenderness (especially in RUQ), guarding, or masses
• Mental status: watch for confusion, disorientation (especially in elderly or severe sepsis)

3.Laboratory and Diagnostic Monitoring

• Liver function tests (ALT, AST, ALP, GGT, bilirubin)
• White blood cell count and inflammatory markers (CRP, ESR, procalcitonin)
• Blood cultures before antibiotic therapy
• Imaging: ultrasound, CT scan, or MRCP to confirm diagnosis and locate obstruction

Nursing Diagnoses

Possible nursing diagnoses for patients with cholangitis include:

• Acute pain related to inflammation of the biliary tract
• Risk for infection/sepsis related to ascending biliary infection
• Ineffective thermoregulation related to infection
• Impaired skin integrity (secondary to pruritus and jaundice)
• Deficient fluid volume related to fever, vomiting, and decreased oral intake
• Anxiety related to hospitalization and illness

Nursing Interventions

1.Monitoring and Assessment

• Frequent monitoring of vital signs (especially temperature, heart rate, blood pressure, and respiratory rate)
• Monitor mental status and signs of sepsis or shock
• Track fluid balance: intake and output, monitor for signs of dehydration or fluid overload
• Assess pain level regularly and evaluate response to interventions
• Monitor skin integrity and assess for pressure areas, pruritic lesions, or breakdown

2.Pain Management

• Administer prescribed analgesics (usually paracetamol or opioids, as indicated)
• Non-pharmacological techniques: positioning for comfort, relaxation techniques, applying warm compresses (if appropriate)
• Communicate with the medical team if pain is not controlled or worsens

3.Infection Control and Antibiotic Stewardship

• Start IV antibiotics as ordered, ideally after blood cultures are drawn
• Maintain strict aseptic technique with all invasive procedures and IV lines
• Monitor for signs of antibiotic reaction or complications
• Educate the patient and family about the importance of completing the antibiotic course

4.Fluid and Electrolyte Management

• Administer IV fluids to maintain adequate hydration and perfusion
• Monitor electrolyte levels and correct imbalances as needed
• Observe for signs of fluid overload (edema, crackles, elevated jugular venous pressure) especially in elderly or those with cardiac/renal compromise

5.Nutritional Support

• Assess nutritional status and consult with dietitian as necessary
• If oral intake is tolerated, provide small, frequent, low-fat meals to decrease biliary stimulation
• For severe cases or those unable to eat, consider enteral or parenteral nutrition under medical guidance

6.Skin Care

• Maintain skin hygiene, especially for jaundiced or pruritic skin
• Use mild soaps, moisturizers, and keep nails short to reduce scratching
• Apply topical anti-pruritic agents if prescribed
• Reposition frequently to prevent pressure injuries

7.Patient Comfort and Psychosocial Support

• Provide reassurance and clear explanations of procedures and treatments
• Allow family involvement as appropriate
• Address anxieties and provide resources for emotional support

8.Preparation for Procedures

• Prepare patients for potential interventions such as Endoscopic Retrograde Cholangiopancreatography (ERCP), percutaneous drainage, or surgery
• Ensure informed consent is obtained
• Monitor post-procedure for complications such as bleeding, pancreatitis, or perforation

9.Education and Discharge Planning

• Educate about the signs of recurrence (fever, pain, jaundice) and when to seek medical attention
• Instruction on medication management, wound care (if applicable), and dietary modifications
• Arrange for follow-up appointments and provide contact information for ongoing care
• Discuss lifestyle modifications to reduce recurrence risk (weight management, reducing alcohol intake, and managing gallstone risk factors)

Collaborative Care

Nurses should collaborate with:

• Physicians for timely review and management of antibiotic therapy, procedural interventions, and overall medical plan
• Dietitians for nutritional assessment and support
• Pharmacists for medication reconciliation, interactions, and stewardship
• Social workers and counselors for psychosocial needs

Special Considerations

• Elderly patients may present atypically, often with confusion or general malaise rather than classic symptoms. Close monitoring is essential.
• Immunocompromised patients are at higher risk for severe infection and complications.
• Pediatric cases are rare but require age-appropriate care and involvement of caregivers in education and monitoring.

Prevention and Health Promotion

• Educate high-risk patients about gallstone prevention and early symptom recognition
• Encourage routine check-ups and timely management of biliary disease
• Stress the importance of adherence to prescribed treatments and follow-up care

Documentation

Accurate and timely documentation is essential to ensure continuity and quality of care. Key areas to document include:

• Vital signs and physical findings
• Pain assessment and management
• Medication and fluid administration
• Patient responses and progress
• Education and discharge instructions

REFERENCES

1. Merck Manual Profession Version. Choledocholithiasis and Cholangitis. https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/gallbladder-and-bile-duct-disorders/choledocholithiasis-and-cholangitis. Reviewed/Revised 8/2023.
2. Mohammad Alizadeh AH. Cholangitis: Diagnosis, Treatment and Prognosis. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5719198/. J Clin Transl Hepatol. 2017 Dec 28;5(4):404-413.
3. Kiriyama S, Kozaka K, Takada T, et al. Tokyo guidelines 2018: diagnostic criteria and severity grading of acute cholangitis (with videos). J Hepatobiliary Pancreat Sci. 2018 Jan;25(1):17-30.
4. Virgile J, Marathi R. Cholangitis . https://www.ncbi.nlm.nih.gov/books/NBK558946/. 2023 Jul 3. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan.
5. Ami TR. Allscripts EPSi. Mayo Clinic. Aug. 8, 2023.
6. Feldman M, et al., eds. Primary biliary cholangitis. In: Sleisenger and Fordtran’s Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 11th ed. Elsevier; 2021. https://www.clinicalkey.com.

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