CHOLANGITIS

Cholangiohepatitis Syndrome

Definition:
An inflamed bile duct and liver, more commonly seen in cats

Signs:
Reduced appetite, abdominal pain, fever, vomiting, jaundice (skin goes yellow). Signs can be
sudden or long term

Advice:
Causes can be inflammatory, cancer, bacterial infection and unknown. Bacterial infection can
often be cured but inflammatory often recurs. Test may determine the cause. Hospitalisation is
not unusual management

OVERVIEW
• The liver is the largest gland in the body; it has many functions, including production of bile (a
fluid substance involved in digestion of fats); bile ducts begin within the liver itself as tiny
channels to transport bile—the ducts join together to form larger bile ducts and finally enter
the extrahepatic or common bile duct, which empties into the upper small intestine; the system
of bile ducts is known as the “biliary tree”
• The gallbladder is the storage unit for bile; bile is stored until it is needed for fat digestion
• “Cholangitis” is inflammation of the bile duct or the biliary tree
• “Cholangiohepatitis” is inflammation of biliary structures and surrounding liver tissue
• Cholangitis/cholangiohepatitis syndrome occurs more commonly in cats; it is classified as
“suppurative” or “nonsuppurative” (lymphoplasmacytic, lymphocytic), “granulomatous,” or
“lymphoproliferative” (transition to lymphoma) based on microscopic examination of biopsy
samples
• “Suppurative” refers to the presence of pus in the affected tissue; “nonsuppurative” refers to an
inflammatory process that is not characterized by the presence of pus—in
cholangitis/cholangiohepatitis syndrome, the inflammatory process is characterized by the
presence of lymphocytes and plasma cells (so-called “lymphoplasmacytic” disease) or
lymphocytes (so-called “lymphocytic” disease); lymphocytes are a type of white blood cell that
are formed in lymphatic tissues throughout the body—lymphocytes are involved in the immune
process; plasma cells are specialized white blood cells; plasma cells are lymphocytes that have
been altered to produce immunoglobulin, an immune protein or antibody necessary for fighting
disease

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 • “Granulomatous” refers to nodular, inflammatory lesions; “lymphoproliferative” refers to
conditions in which an excessive number of lymphocytes are produced; “lymphoma” is a type
of cancer that develops from lymphoid tissue, including lymphocytes, a type of white blood cell
formed in lymphatic tissues throughout the body

SIGNALMENT/DESCRIPTION OF PET
Species
• Cats (common)
• Dogs (uncommon)

Breed Predilections
• Cats—possibly Himalayan, Persian, and Siamese

Mean Age and Range

• Suppurative cholangitis/cholangiohepatitis syndrome—range, 0.4–16 years of age; mostly
young to middle-aged cats
• Nonsuppurative cholangitis/cholangiohepatitis syndrome—range, 2–17 years of age; mostly
middle-aged cats

Predominant Sex
• Suppurative cholangitis/cholangiohepatitis syndrome—male cats more likely to be affected
than female cats
• Nonsuppurative cholangitis/cholangiohepatitis syndrome—none

SIGNS/OBSERVED CHANGES IN THE PET

• Suppurative cholangitis/cholangiohepatitis syndrome—most severe clinical illness characterized
by “acute abdomen” (sudden onset of severe abdominal pain), fever; often less than 5 days in
duration; associated with blockage of the extrahepatic or common bile duct (known as
“extrahepatic bile duct obstruction”) May also see lack of appetite (known as “anorexia”);
vomiting; may have yellowish discoloration to the gums and other tissues of the body (known as
“jaundice” or “icterus”); dehydration; collapse; generalized bacterial infection (known as
“sepsis”); shock
• Nonsuppurative cholangitis/cholangiohepatitis syndrome—illness of greater than 3 weeks in
duration (may have signs of illness for months to years); cyclic illness; long-term (chronic) vague
signs: sluggishness (lethargy), vomiting, lack of appetite (anorexia), and weight loss; few
physical abnormalities other than enlarged liver (known as “hepatomegaly”); thickened
intestines with inflammatory bowel disease (IBD); variable yellowish discoloration to the gums

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 and other tissues of the body (jaundice or icterus); rare fluid buildup in the abdomen (known as
“abdominal effusion” or “ascites”)
• Decreased number of bile ducts (known as “ductopenia”) can be associated with
nonsuppurative cholangitis/cholangiohepatitis syndrome in cats—causes increased appetite
(known as “polyphagia”) due to reduced bile flow with poor digestion of fats and presence of
large amounts of fat in the stool (known as “steatorrhea”), due to the inability to digest the fat,
and leading to decreased levels of fat-soluble substances (such as vitamin K1, essential fatty
acids, vitamin E); variable pale or grayish coloration to the stools (known as “acholic feces”),
due to the lack of bile pigments that cause the normal brown color of bowel movements
• Unkempt coat, variable hair loss on the sides of the chest

CAUSES
Suppurative Cholangitis/Cholangiohepatitis Syndrome

• Bacterial infection—most common in cats: E. coli, Enterobacter, Enterococcus, β-hemolytic

Streptococcus, Klebsiella, Actinomyces, Clostridia, and Bacteroides; also rarely associated with
toxoplasmosis; dogs: intestinal bacterial opportunists (bacteria that usually do not cause
disease, but are able to cause disease because the pet's body and/or immune system has been
weakened by some other disease process); rare cases with Campylobacter, Salmonella, and
Leptospira, others
• Condition may be seen following blockage of the extrahepatic or common bile duct
(extrahepatic bile duct obstruction) and diseases in which flow of bile is decreased or stopped
(known as “cholestasis”)

Nonsuppurative Cholangitis/Cholangiohepatitis Syndrome

• Co-existent disorders—inflammation of the gallbladder (known as “cholecystitis”); presence of

hard, solid material in the bile duct or gallbladder (known as “cholelithiasis”); inflammation of
the pancreas (known as “pancreatitis”); blockage of the extrahepatic or common bile duct
(extrahepatic bile duct obstruction); inflammatory bowel disease; long-term (chronic)
inflammation of the tissue spaces in the kidneys (known as “chronic interstitial nephritis”) in
cats

RISK FACTORS
• Suppurative cholangitis/cholangiohepatitis syndrome—blockage of the extrahepatic or common
bile duct (extrahepatic bile duct obstruction); diseases in which flow of bile is decreased or
EVH Emergency Vet Hospital Chokolich Street WANGARA WA 6065 T 92004460 E [email protected]
 stopped (cholestasis); infections elsewhere in the body
• Nonsuppurative cholangitis/cholangiohepatitis syndrome in cats—inflammatory bowel disease;
inflammation of the pancreas (pancreatitis); blockage of the extrahepatic or common bile duct
(extrahepatic bile duct obstruction); possibly long-term (chronic) inflammation of the tissue
spaces in the kidneys (chronic interstitial nephritis)
• Genetic tests for feline polycystic disease may be appropriate

TREATMENT
HEALTH CARE
Inpatient Management

• Suppurative cholangitis/cholangiohepatitis syndrome with sudden (acute) illness characterized

by fever, painful abdomen, abnormal white blood cell count and cell immaturity (left shift)—
hydration support; pre-surgery antibiotics; if the pet has blockage of the extrahepatic or
common bile duct (extrahepatic bile duct obstruction) or inflammation of the gallbladder
(cholecystitis); continue antibiotics for at least 4–8 weeks; treat with medications (such as
ursodeoxycholic acid, SAMe) to improve the secretion of bile until liver enzyme values
normalize on bloodwork (serum biochemistry profile) or signs resolve
• Nonsuppurative cholangitis/cholangiohepatitis syndrome in cats with clinical signs of disease—
fluid therapy, as necessary; diagnostic evaluations; liver biopsy (the veterinarian will administer
vitamin K1 before liver biopsy)
• Both forms of cholangitis/cholangiohepatitis syndrome in cats—may need blood transfusion
following surgery or biopsy
• Supplement fluids with B vitamins, potassium chloride, and potassium phosphate, as needed

Outpatient Management

• Suppurative cholangitis/cholangiohepatitis syndrome—after sudden (acute) crisis has been

managed
• Nonsuppurative cholangitis/cholangiohepatitis syndrome—after resolution of sudden (acute)
crisis, provide lifelong treatment to alter the immune system (known as “immune-system
modulation”) as well as antioxidant and liver-protective therapy

ACTIVITY
• Restricted while the pet has clinical signs of disease

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DIET
• Nutritional support—to avoid hepatic lipidosis, feed a balanced high-protein, high-calorie feline
diet; supplement water-soluble vitamins (such as vitamin B); “hepatic lipidosis” is a disease in
which fats and lipids (compounds that contain fats or oils) accumulate in the liver as a possible
complication of lack of appetite (anorexia)
• Antigen-restricted diet with co-existent inflammatory bowel disease
• Fat-restricted diet, if the pet has severe decrease in bile ducts (ductopenia), abnormal
absorption of fat (known as “fat malabsorption”), or long-term (chronic) inflammation of the
pancreas (pancreatitis) causing abnormal digestion of food (known as “maldigestion”)
• May require feeding tubes; rarely requires feeding through intravenous fluids (known as
“parenteral nutrition”)

SURGERY
• Surgical removal of the gallbladder (known as “cholecystectomy”)—if the pet has inflammation
of the gallbladder (cholecystitis)
• Surgical procedure to produce a new connection between the biliary tree and the small
intestines (known as “cholecystoenterostomy”)—may be needed in pets with blockage of the
extrahepatic or common bile duct (extrahepatic bile duct obstruction)
• Surgical removal of stones in the gallbladder (stones known as “choleliths”)

MEDICATIONS
Medications presented in this section are intended to provide general information about possible
treatment. The treatment for a particular condition may evolve as medical advances are made; therefore,
the medications should not be considered as all-inclusive

Antibiotics for Suppurative Cholangitis/Cholangiohepatitis Syndrome

• Antibiotics that kill bacteria (known as “bactericidal antibiotics”)—against intestinal bacterial

opportunists (bacteria that usually do not cause disease, but are able to cause disease because
the pet's body and/or immune system has been weakened by some other disease process);
may include ticarcillin, amoxicillin-clavulanic acid or enrofloxacin combined with metronidazole
• Resistant enterococci—vancomycin; “enterococci” are bacteria normally found in bowel
movement of animals and people that occasionally cause disease
• Modify antibiotics, based on bacterial culture and sensitivity reports

Medications to Alter the Immune Response (Immunomodulation) for Nonsuppurative

Cholangitis/Cholangiohepatitis Syndrome

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 • Steroids—prednisolone; long-term (chronic) therapy usually needed, tapered to the lowest
effective dose
• Metronidazole—in combination with prednisolone, especially if the pet has co-existent
inflammatory bowel disease, recent work confirms use of metronidazole in nonsuppurative
form of feline CCHS
• Cats with confirmed decreased number of bile ducts (ductopenia) require more aggressive
treatment; clinical experience suggests combination of prednisolone, metronidazole; provide
folate (folinic acid) with pulsed chlorambucil or methotrexate
• Some cats require chemotherapy protocols developed for intestinal lymphoma (“lymphoma” is
a type of cancer that develops from lymphoid tissue, including lymphocytes, a type of white
blood cell formed in lymphatic tissues throughout the body)

Antioxidants
• Vitamin E (tocopherol)—higher dose if the pet has long-term (chronic) blockage of the
extrahepatic or common bile duct (extrahepatic bile duct obstruction) or decrease in the
number of bile ducts (ductopenia) because of abnormal absorption of fat (fat malabsorption)
• S-adenosylmethionine (SAMe) enteric coated prior to meals

Other
• Ursodeoxycholic acid (given with food)—has numerous potentially beneficial effects, including
altering the immune response (immunomodulation), protecting the liver, causing secretion of
bile, and providing antioxidant effects, not appropriate in all cases, so biopsy helpful in cats
before prescribing
• Taurine supplement
• B-vitamin supplementation with thiamine (B1) and B12

FOLLOW-UP CARE
PATIENT MONITORING
• Nonsuppurative cholangitis/cholangiohepatitis syndrome—initially, monitor bloodwork (liver
enzyme and bilirubin levels) every 7–14 days; with remission, assess bloodwork quarterly

PREVENTION AND AVOIDANCE

• Control inflammatory bowel disease

POSSIBLE COMPLICATIONS
• Suppurative cholangitis/cholangiohepatitis syndrome may transform into nonsuppurative

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 cholangitis/cholangiohepatitis syndrome or sclerosing cholangitis/cholangiohepatitis syndrome
(characterized by thickening or hardening of the biliary and/or liver tissues)
• Diabetes mellitus may occur in 30% of cats with sclerosing cholangitis/cholangiohepatitis
syndrome treated with prednisolone
• Hepatic lipidosis may develop with inadequate nutritional intake and in some cats, with steroid
therapy; “hepatic lipidosis” is a disease in which fats and lipids (compounds that contain fats or
oils) accumulate in the liver of cats may occur if inadequate food intake or as a steroid side
effect

EXPECTED COURSE AND PROGNOSIS

• Suppurative cholangitis/cholangiohepatitis syndrome—may be cured
• Nonsuppurative cholangitis/cholangiohepatitis syndrome—long-term (chronic) disease; long-
term remission possible (remission greater than 8 years has been documented)

KEY POINTS
• ”Cholangitis” is inflammation of the bile duct or the biliary tree
• “Cholangiohepatitis” is inflammation of biliary structures and surrounding liver tissue
• Cholangitis/cholangiohepatitis syndrome occurs primarily in cats; it is classified as
“suppurative” or “nonsuppurative” (lymphoplasmacytic, lymphocytic), “granulomatous,” or
“lymphoproliferative” based on microscopic examination of biopsy samples
• Suppurative cholangitis/cholangiohepatitis syndrome—sudden (acute) illness; fever; lack of
appetite (anorexia); vomiting; painful abdomen; may have yellowish discoloration to the gums
and other tissues of the body (jaundice or icterus); dehydration; collapse; shock
• Nonsuppurative cholangitis/cholangiohepatitis syndrome—illness of greater than 3 weeks in
duration (may have signs of illness for months to years); cyclic illness; long-term (chronic) vague
signs: sluggishness (lethargy), vomiting, lack of appetite (anorexia), and weight loss; few
physical abnormalities other than enlarged liver (hepatomegaly); thickened intestines if
associated with inflammatory bowel disease; variable yellowish discoloration to the gums and
other tissues of the body (jaundice or icterus); rare fluid buildup in the abdomen (ascites)
• Long-term (chronic) nature of nonsuppurative cholangitis/cholangiohepatitis syndrome
requires lifelong therapy

Blackwell’s Five-Minute Veterinary Consult: Canine and Feline, Sixth Edition, Larry P. Tilley and Francis
W.K. Smith, Jr. © 2015 John Wiley & Sons, Inc.

EVH Emergency Vet Hospital Chokolich Street WANGARA WA 6065 T 92004460 E [email protected]