Type of Cholangitis
Cholangitis encompasses various inflammatory conditions affecting the bile ducts. Primary Sclerosing Cholangitis (PSC), a chronic liver disease often associated with inflammatory bowel diseases, leads to bile duct inflammation and strictures. Bacterial Cholangitis, Recurrent Pyogenic Cholangitis (RPC), and Parasitic Cholangitis are other forms of cholangitis, each with distinct causes and characteristics. Diagnosis involves imaging and biopsy. PSC, if untreated, can progress to cirrhosis and bile duct cancer. Treatment options include medical therapies like ursodiol and, in severe cases, liver transplantation. Early detection and intervention are critical for managing these complex cholangitis conditions.
| Aspect | Primary Sclerosing Cholangitis (PSC) | Bacterial Cholangitis | Recurrent Pyogenic Cholangitis (RPC) | Parasitic Cholangits |
|---|---|---|---|---|
| Etiology | Unknown; Various Theories Including Immunoregulatory Abnormalities | 1. Obstructive etiology (CBD calculus, instrumentation of the biliary tree, malignant disease, sclerosing cholangitis, periampullary diverticula and Malignancies) 2. Partial obstruction has higher chances of biliary bacterial contamination | 1. Recurrent Bacterial Infection | 1. Echinococcosis sp. 2. Clonorchiasis 3. Opisthorchiasis sp. 4. Fascioliasis 5. Ascariasis 6. Schistosomiasis |
| Pathophysiology | Stage 1: findings limited to portal triads - Degeneration of epithelial cells in the bile duct and by - Infiltration of the bile duct by lymphocytes and neutrophils. - Inflammation, scarring, and enlargement of the portal triads with occasional portal edema. Stage 2: fibrosis and inflammation infiltrate the periportal parenchyma, where they gradually destroy periportal hepatocytes - Periductal fibrosis called “onion-skin” Stage 3: portal-to-portal fibrous septa form. Bile ducts disappear or undergo severe degenerative changes. Stage 4: End stage, is characterised by cirrhosis | Increased Intra-biliary pressure (≥ 20 cm H2O) causes: - Bile stagnation leading to bacterial infection and calculi formation - Increased permeability of biliary ducts causing toxin entry into blood stream - Extension of inflammatory process in periportal, peribiliary tissue (causing increased arterial flow and dilatation of peribiliary venous plexus). - Reduced production of intra-biliary IgA causing increased small gut microflora | 1. Biliary tract ectasia 2. Extension of inflammatory process in periportal, peribiliary tissue causing fibrosis | Obstructive |
| Clinical symptoms | 1. Generally asymptomatic (abnormal LFTs are the only abnormal finding) 2. Strong association with inflammatory bowel disease (Ulcerative colitis >> Crohn's disease) 3. Right upper quadrant pain, pruritus, jaundice and fatigue 4. Fever with chills can occur if there is added infection | 1. Charcot triad (Pain, Fever, Jaundice) 2. Reynolds pentad (Charcot Traid + Lethargy and shock) | Chronic on and off epigastric Pain, fever and jaundice | Silent, subacute or can can present with acute features depending on the causative agent |
| Biliary channels | Both intra- and extrahepatic bile ducts are involved - alternate dilatation and narrowing of biliary ducts | 1. Intrahepatic Biliary ducts - diffuse / segmental dilatation, initially central then peripheral channels 2. Extrahepatic Biliary ducts - CBD dilatation in patients with choledocholithiasis | Disproportionate dilatation of peripheral vs central biliary ducts. 1. Peripheral ducts - stenosis, stricture, decreased branching and abrupt tapering 2. Central and Extrahepatic ducts - dilatation and calculi formation | 1. Peripheral duct dilation 2. Filling defect (Parasites) |
| Radiologic Findings (MRCP sequencing is recommended) | 1. Beaded appearance involving both intra- and extrahepatic bile ducts - alternate dilatation and narrowing (short and long length stricture depending on subtype) of biliary ducts 2. Diverticular outpouching 3. Pruned-tree appearance at chronic stage - advance fibrosis of peripheral ducts 4. Hepaticolith formation 5. Mural nodules and shaggy margins can be present | 1. Smooth and symmetrical dilatation of proximal biliary ducts (proximal to site of etiology) 2. Parenchymal changes (T2W and T1C+) wedge shaped, peripheral patchy inhomogeneous and peribiliary distribution 3. Intensely enhancing, enlarged and bulging papilla 4. Liver abscess communicating with biliary channel 5. Portal vein thrombosis | 1. Arrowhead appearance - Peripheral ducts with stenosis, stricture, decreased branching and abrupt tapering 2. Intrahepatic biliary calculi - T1W (hyperintense) and T2W (Hypointense). Better visualised on NCCT. 3. Parenchymal atrophy - segmental / lobar in chronic cases 4. Liver abscesses, Portal vein thrombosis 5. Cholangiocarcinoma - especially in areas of parenchymal atrophy 6. USG helps in follow up and routine assessment | 1. Enhancing biliary channel wall 2. Obstructive features may or may not be present |
| Diagnostic Criteria and Lab investigations | 1. Cholangiography 2. Elevated Liver Enzymes - elevated levels of serum alkaline phosphatase (> 6 months is diagnostic) or g-glutamyl transferase. - Serum bilirubin level persistently > 1.5 mg/ dl is a sign of a poor prognosis and indicate irreversible, medically untreatable disease. _ Serum ALP levels > 1.5 times the upper limit poor outcome 3. CA 19-9 - suspicion for cholangiocarcinoma 4. Image guided Liver biopsy | 1. CBC, LFTs 2. Blood culture (usually negative) - perform prior to start of antibiotic management 3. If PTBD drainage is performed small sample of bile is collected for lab test (gram staining, routine culture and sensitivity) | Clinical, Imaging Findings, History of recurrent infections | 1. Disease specific serology (IgE antibody detection) 2. Eosinophilia 3. LFTs 4. Imaging study |
| Complications | 1. Biliary stricture 2. Recurrent Sclerosing cholangitis 3. Portal vein thrombosis leading to lobar and segmental atrophy 4. Biliary cirrhosis 2. Portal Hypertension 5. Cholangiocarcinoma (new onset of jaundice, fever, or weight loss, or significant changes in LFT) | Acute Complications: 1. Sepsis (predominately gram negative rods, however can be polymicrobial) 2. Hepatic abscesses (Cholangiolar abscess) 3. Portal vein thrombosis 4. Biloma and Bile peritonitis Chronic Complications: 1. Biliary stricture 2. Recurrent Sclerosing cholangitis 3. Portal vein thrombosis leading to lobar and segmental atrophy 4. Cholangiocarcinoma | 1. Biliary fibrosis and stricture 2. Intrahepatic calculi formation 3. Portal vein thrombosis leading to lobar and segmental atrophy (left lobe > right posterior segments > right anterior segments) 4. High risk of Cholangiocarcinoma | 1. Bacterial Cholangitis 2. Recurrent Sclerosing cholangitis 3. Cholangiocarcinoma |
| Treatment Options | 1. Antibiotic therapy (Broad-spectrum antibiotics like piperacillin plus tazobactam) x 7-10 days - if patient is non-responsive after medical management biliary decompression might be needed. 2. Biliary decompression (especially patients with liver abscess)- ERCP stenting / PTBD drainage 3. Biliary calculi and stricture - Multidisciplinary team of Interventional radiologist, Gastroenterologist and GI surgery - ERCP guided stone extraction using a balloon, basket, forceps - Percutaneous hepaticolith extraction in patients with previous Roux-en-Y surgery -Stricture dilatation using balloon catheters 4. Surgical management: Stone extraction, choledochojejunostomy, Lobar or segmental resection, Biliary bypass surgery, Liver transplant (~ 35-40% patients of PSC). 5. Pruritus - Cholestyramine 4-8 mg x BD/TDS (2-4 days to show results). Other drugs Colestipol hydrochloride. 6. Assessment for Fat-soluble vitamins (A > K > D and E)and their replacement 7. Ursodiol improves LFTs (however use is not recommended by all societies) | 1. Antibiotic therapy (Broad-spectrum antibiotics like piperacillin plus tazobactam) x 7-10 days - if patient is non-responsive biliary decompression is needed. 2. Biliary decompression (especially patients with liver abscess)- ERCP stenting / PTBD drainage | 1. Management of Acute episodes similar to Bacterial Cholangitis 2. Biliary calculi and stricture - Multidisciplinary team of Interventional radiologist, Gastroenterologist and GI surgery - ERCP guided stone extraction using a balloon, basket, forceps - Percutaneous hepaticolith extraction in patients with previous Roux-en-Y surgery -Stricture dilatation using balloon catheters 3. Surgical management: Stone extraction, choledochojejunostomy, Lobar or segmental resection, Biliary bypass surgery. 4. Biliary decompression (especially patients with liver abscess) 5. Pruritus - Cholestyramine 4-8 mg x BD/TDS (2-4 days to show results). Other drugs Colestipol hydrochloride. 6. Assessment for Fat-soluble vitamins (A > K > D and E)and their replacement | 1. Parasite specific anti-parasitic medication 2. Surgery |
| Follow-up | 1. Patients without inflammatory bowel disease should have a colonoscopy every few years due to their increased risk of colonic lesions. 2. An annual ultrasonographic examination of the gallbladder is recommended to check for polyps or mass lesions. 3. Patients with gallbladder masses of any size should undergo cholecystectomy due to the risk of cancer. |
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