A person with cholangitis may complain of abdominal pain (particularly in the right upper quadrant of the abdomen), fever, rigors (uncontrollable shaking) and a feeling of uneasiness (malaise). Some may report jaundice (yellow discoloration of the skin and the whites of the eyes).

Physical examination findings typically include jaundice and right upper quadrant tenderness. Charcot's triad is a set of three common findings in cholangitis: abdominal pain, jaundice, and fever. This was assumed in the past to be present in 50–70% of cases, although more recently the frequency has been reported as 15–20%. Reynolds' pentad includes the findings of Charcot's triad with the presence of septic shock and mental confusion. This combination of symptoms indicates worsening of the condition and the development of sepsis, and is seen less commonly still.

In the elderly, the presentation may be atypical; they may directly collapse due to sepsis without first showing typical features. Those with an indwelling stent in the bile duct (see below) may not develop jaundice.

Most people with gallstones do not have symptoms. When a gallstone lodges in the cystic duct, they experience biliary colic. Biliary colic is abdominal pain in the right upper quadrant or epigastric region. It is episodic, occurs after eating greasy or fatty foods, and leads to nausea and/or vomiting. People who suffer from cholecystitis most commonly have symptoms of biliary colic before developing cholecystitis. The pain becomes more severe and constant in cholecystitis. Nausea is common and vomiting occurs in 75% of people with cholecystitis. In addition to abdominal pain, right shoulder pain can be present.

On physical examination, fever is common. A gallbladder with cholecystitis is almost always tender to touch. Because of the inflammation, its size can be felt from the outside of the body in 25–50% of people with cholecystitis. Pain with deep inspiration leading to termination of the breath while pressing on the right upper quadrant of the abdomen usually causes pain (Murphy's sign). Murphy's sign is sensitive, but not specific for cholecystitis. Yellowing of the skin (jaundice) may occur but is often mild. Severe jaundice suggests another cause of symptoms such as choledocholithiasis. People who are old, have diabetes, chronic illness, or who are immunocompromised may have vague symptoms that may not include fever or localized tenderness.

Ascending cholangitis, also known as acute cholangitis or simply cholangitis, is an infection of the bile duct (cholangitis), usually caused by bacteria ascending from its junction with the duodenum (first part of the small intestine). It tends to occur if the bile duct is already partially obstructed by gallstones.

Cholangitis can be life-threatening, and is regarded as a medical emergency. Characteristic symptoms include yellow discoloration of the skin or whites of the eyes, fever, abdominal pain, and in severe cases, low blood pressure and confusion. Initial treatment is with intravenous fluids and antibiotics, but there is often an underlying problem (such as gallstones or narrowing in the bile duct) for which further tests and treatments may be necessary, usually in the form of endoscopy to relieve obstruction of the bile duct. The word is from Greek "chol"-, bile + "ang"-, vessel + -"itis", inflammation.

A number of complications may occur from cholecystitis if not detected early or properly treated. Signs of complications include high fever, shock and jaundice. Complications include the following:

- Gangrene

- Gallbladder rupture

- Empyema

- Fistula formation and gallstone ileus

- Rokitansky-Aschoff sinuses

Pain is the most common presenting symptom. It is usually described as sharp right upper quadrant pain that radiates to the right shoulder, or less commonly, behind the breastbone. Nausea and vomiting can be associated with biliary colic. Individuals may also present with pain that is induced following a fatty meal and the symptom of indigestion. The pain often lasts longer than 30 minutes, up to a few hours.

Patients usually have normal vital signs with biliary colic, whereas patients with cholecystitis are usually febrile and more ill appearing. Lab studies that should be ordered include a complete blood count, liver function tests and lipase. In biliary colic, lab findings are usually within normal limits. Alanine aminotransferase and aspartate transaminase are usually suggestive of liver disease whereas elevation of bilirubin and alkaline phosphatase suggests common bile duct obstruction. Pancreatitis should be considered if the lipase value is elevated; gallstone disease is the major cause of pancreatitis.

Gallstones may be asymptomatic, even for years. These gallstones are called "silent stones" and do not require treatment. The size and number of gallstones present does not appear to influence whether people are symptomatic or asymptomatic. A characteristic symptom of gallstones is a gallstone attack, in which a person may experience colicky pain in the upper-right side of the abdomen, often accompanied by nausea and vomiting, that steadily increases for approximately 30 minutes to several hours. A person may also experience referred pain between the shoulder blades or below the right shoulder. These symptoms may resemble those of a "kidney stone attack". Often, attacks occur after a particularly fatty meal and almost always happen at night, and after drinking.

In addition to pain, nausea, and vomiting, a person may experience a fever. If the stones block the duct and cause bilirubin to leak into the bloodstream and surrounding tissue, there may also be jaundice and itching. This can also lead to confusion. If this is the case, the liver enzymes are likely to be raised.

Presentation can be atypical with no pain or fever especially in the elderly population. Hepatolithiasis may present with biliary colic, acute pancreatitis, obstructive jaundice and less commonly, hepatomegaly and abnormal liver chemistry. Chronic biliary obstruction may cause jaundice, pruritus, liver abscess, and liver atrophy, mostly affecting the left lobe and the left lateral segment of the liver, and eventually secondary biliary cirrhosis and cholangiocarcinoma.

Murphy's sign is commonly negative on physical examination in choledocholithiasis, helping to distinguish it from cholecystitis. Jaundice of the skin or eyes is an important physical finding in biliary obstruction. Jaundice and/or clay-colored stool may raise suspicion of choledocholithiasis or even gallstone pancreatitis. If the above symptoms coincide with fever and chills, the diagnosis of ascending cholangitis may also be considered.

Greater than 70% of people with gallstones are asymptomatic and are found incidentally on ultrasound. Studies have shown that 10% of those people will develop symptoms within five years of diagnosis and 20% within 20 years.

Gallstone disease refers to the condition where gallstones are either in the gallbladder or common bile duct. The presence of stones in the gallbladder is referred to as cholelithiasis, from the Greek "chol"- (bile) + "lith"- (stone) + -"iasis" (process). If gallstones migrate into the ducts of the biliary tract, the condition is referred to as choledocholithiasis, from the Greek "chol"- (bile) + "docho"- (duct) + "lith"- (stone) + "iasis"- (process). Choledocholithiasis is frequently associated with obstruction of the biliary tree, which in turn can lead to acute ascending cholangitis, from the Greek: "chol"- (bile) + "ang"- (vessel) + "itis"- (inflammation), a serious infection of the bile ducts. Gallstones within the ampulla of Vater can obstruct the exocrine system of the pancreas, which in turn can result in pancreatitis.

Suppurative cholangitis, liver abscess, empyema of the gallbladder, acute pancreatitis, thrombophlebitis of hepatic or portal veins, and septicemia are acute complications of the disease, to which patients may succumb during the acute attacks.

Chronically, complications include cholangiocarcinoma and intraductal papillary neoplasm.

Biliary sludge may cause complications such as biliary colic, acute cholecystitis, acute cholangitis, and acute pancreatitis.

Biliary colic, also known as a gallbladder attack or gallstone attack, is when pain occurs due to a gallstone temporarily blocking the bile duct. Typically, the pain is in the right upper part of the abdomen, and it can radiate to the shoulder. Pain usually lasts from one to a few hours. Often, it occurs after eating a heavy meal, or during the night. Repeated attacks are common.

Gallstone formation occurs from the precipitation of crystals that aggregate to form stones. The most common form is cholesterol gallstones. Other forms include calcium, bilirubin, pigment, and mixed gallstones. Other conditions that produce similar symptoms include appendicitis, stomach ulcers, pancreatitis, and gastroesophageal reflux disease.

Treatment for gallbladder attacks is typically surgery to remove the gallbladder. This can be either done through small incisions or through a single larger incision. Open surgery through a larger incision is associated with more complications than surgery through small incisions. Surgery is typically done under general anesthesia. In those who are unable to have surgery, medication to try to dissolve the stones or shock wave lithotripsy may be tried. , it is not clear whether surgery is indicated for everyone with biliary colic.

In the developed world, 10-15% of adults have gallstones. Of those with gallstones, biliary colic occurs in 1–4% each year. Nearly 30% of people have further problems related to gallstones in the year following an attack. About 15% of people with biliary colic eventually develop inflammation of the gallbladder if not treated. Other complications include inflammation of the pancreas.

Common bile duct stone, also known as choledocholithiasis, is the presence of gallstones in the common bile duct (thus "" + ""). This condition causes jaundice and liver cell damage. Treatment is by cholecystectomy and ERCP.

Primary sclerosing cholangitis is typically classified into three subgroups based on whether the small and/or large bile ducts are affected. The subgroups of PSC include the following:

- Classic PSC

- Small-duct PSC

- PSC associated with autoimmune hepatitis

Nearly half of people with PSC do not have symptoms and are often incidentally discovered to have PSC due to abnormal liver function tests, but a substantial proportion will have debilitating signs and symptoms of the disease. Signs and symptoms of PSC may include severe itching and non-specific fatigue. Yellowing of the skin and white portion of the eyes may also be seen. Enlargement of the liver and spleen are seen in approximately 40% of affected individuals. Abdominal pain affects about 20% of people with PSC.

Multiple episodes of life-threatening acute cholangitis (infection within the bile ducts) can be seen due to impaired drainage of the bile ducts, which increases the risk of infection.

- Dark urine due to excess conjugated bilirubin, which is water-soluble and excreted by the kidneys (i.e. choluria)

- Malabsorption, especially of fat, and steatorrhea (fatty stool), due to an inadequate amount of bile reaching the small intestine, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.

- Portal hypertension, a complication of cirrhosis, which can manifest with esophageal and parastomal varices as well as hepatic encephalopathy (mental status alteration/disturbance caused by liver dysfunction and shunting of blood away from the scarred liver; such that ammonia detoxification is reduced with concomitant encephalopathy).

Biliary sludge is typically diagnosed by CT scan or transabdominal ultrasonography. Endoscopic ultrasonography is another more sensitive option. However, the gold standard is considered to be direct microscopy of aspirated gallbladder bile. This method is much more sensitive, although it is less practical.

The most common symptoms and signs include:

- severe epigastric pain (upper abdominal pain) radiating to the back in 50% of cases

- nausea

- vomiting

- loss of appetite

- fever

- chills (shivering)

- hemodynamic instability, including shock

- tachycardia (rapid heartbeat)

- respiratory distress

- peritonitis

- hiccup

Although these are common symptoms, they are not always present. Simple abdominal pain may be the sole symptom.

Signs that are less common, and indicate severe disease, include:

- Grey-Turner's sign (hemorrhagic discoloration of the flanks)

- Cullen's sign (hemorrhagic discoloration of the umbilicus)

- Pleural effusions (fluid in the bases of the pleural cavity)

- Grünwald sign (appearance of ecchymosis, large bruise, around the umbilicus due to local toxic lesion of the vessels)

- Körte's sign (pain or resistance in the zone where the head of pancreas is located (in epigastrium, 6–7 cm above the umbilicus))

- Kamenchik's sign (pain with pressure under the xiphoid process)

- Mayo-Robson's sign (pain while pressing at the top of the angle lateral to the Erector spinae muscles and below the left 12th rib (left costovertebral angle (CVA))

- Mayo-Robson's point – a point on border of inner 2/3 with the external 1/3 of the line that represents the bisection of the left upper abdominal quadrant, where tenderness on pressure exists in disease of the pancreas. At this point the tail of pancreas is projected on the abdominal wall.

- Pandiaraja's sign- ecchymosis of right axilla

Systemic complications include ARDS, multiple organ dysfunction syndrome, DIC, hypocalcemia (from fat saponification), hyperglycemia and insulin dependent diabetes mellitus (from pancreatic insulin-producing beta cell damage), malabsorption due to exocrine failure

- Metabolic

- Respiratory

- Renal

- Renal artery or vein thrombosis

- Renal failure

- Circulatory

- Arrhythmias

- Hypovolemia and shock

- myocardial infarct

- Pericardial effusion

- vascular thrombosis

- Gastrointestinal

- Gastrointestinal hemorrhage from stress ulceration;

- gastric varices (secondary to splenic vein thrombosis)

- Gastrointestinal obstruction

- Hepatobiliary

- Jaundice

- Portal vein thrombosis

- Neurologic

- Psychosis or encephalopathy (confusion, delusion and coma)

- Cerebral Embolism

- Blindness (angiopathic retinopathy with hemorrhage)

- Hematologic

- Anemia

- DIC

- Leucocytosis

- Dermatologic

- Painful subcutaneous fat necrosis

- Miscellaneous

- Subcutaneous fat necrosis

- Arthalgia

Cholestasis is a condition where bile cannot flow from the liver to the duodenum. The two basic distinctions are an obstructive type of cholestasis where there is a mechanical blockage in the duct system that can occur from a gallstone or malignancy, and metabolic types of cholestasis which are disturbances in bile formation that can occur because of genetic defects or acquired as a side effect of many medications.

Under a microscope, the individual hepatocytes will have a brownish-green stippled appearance within the cytoplasm, representing bile that cannot get out of the cell. Canalicular bile plugs between individual hepatocytes or within bile ducts may also be seen, representing bile that has been excreted from the hepatocytes but cannot go any further due to the obstruction. When these plugs occur within the bile duct, sufficient pressure (caused by bile accumulation) can cause them to rupture, spilling bile into the surrounding tissue, causing hepatic necrosis. These areas are known as bile lakes, and are typically seen only with extra-hepatic obstruction.

SSC is thought to develop as a consequence of known injuries or pathological processes of the biliary tree, such as biliary obstruction, surgical trauma to the bile duct, or ischemic injury to the biliary tree. Secondary causes of SSC include intraductal stone disease, surgical or blunt abdominal trauma, intra-arterial chemotherapy, and recurrent pancreatitis. It has been clearly demonstrated sclerosing cholangitis can develop after an episode of severe bacterial cholangitis. Also it was suggested that it can result from insult to the biliary tree by obstructive cholangitis secondary to choledocholithiasis, surgical damage, trauma, vascular insults, parasites, or congenital fibrocystic disorders. Additional causes of secondary SC are toxic, due to chemical agents or drugs.

Pancreatic diseases that affect digestion refers to disorders affecting the exocrine pancreas, which is a part of the pancreas involved in digestion.

One of the most common conditions of the exocrine pancreas is acute pancreatitis, which in the majority of cases relates to gallstones that have impacted in the pancreatic part of the biliary tree, or due to acute or chronic alcohol abuse or as a side-effect of ERCP. Other forms of pancreatitis include chronic and hereditary forms. Chronic pancreatitis may predispose to pancreatic cancer and is strongly linked to alcohol use. Other rarer diseases affecting the pancreas may include pancreatic pseudocysts, exocrine pancreatic insufficiency, and pancreatic fistulas.

Pancreatic disease may present with or without symptoms. When symptoms occur, such as in acute pancreatitis, a person may suffer from acute-onset, severe mid-abdominal pain, nausea and vomiting. In severe cases, pancreatitis may lead to rapid blood loss and systemic inflammatory response syndrome. When the pancreas is unable to secrete digestive enzymes, such as with a pancreatic cancer occluding the pancreatic duct, result in jaundice. Pancreatic disease might be investigated using abdominal x-rays, MRCP or ERCP, CT scans, and through blood tests such as measurement of the amylase and lipase enzymes.

The first symptoms typically include fever, intermittent abdominal pain, and hepatomegaly. Occasionally, jaundice occurs.

Morbidity is common and is caused by complications of cholangitis, sepsis, choledocholithiasis, and cholangiocarcinoma. These morbid conditions often prompt the diagnosis. Portal hypertension may be present, resulting in other conditions including splenomegaly, hematemesis, and melena. These problems can severely affect the patient's quality of life. In a 10-year period between 1995 and 2005, only 10 patients were surgically treated for Caroli disease, with an average patient age of 45.8 years.

After reviewing 46 cases of Caroli disease before 1990, 21.7% of the cases were the result of an intraheptic cyst or nonobstructive biliary tree dilation, 34.7% were linked with congenital hepatic fibrosis, 13% were isolated choledochal cystic dilation, and the remaining 24.6% had a combination of all three.

Ductopenia refers to a reduction in the number of ducts in an organ. It is the histological hallmark of vanishing bile duct syndrome (typically <0.5 bile ducts per portal triad). The most common cause of ductopenia is primary biliary cholangitis.

Other causes of ductopenia include failing liver transplant, Hodgkin's lymphoma, graft-versus-host disease (GVHD), sarcoid, Cytomegalovirus infection, HIV and medication toxicity.

The diagnosis of SSC requires the exclusion of secondary causes of sclerosing cholangitis and recognition of associated conditions that may potentially imitate its classic cholangiographic features. It is morphologically similar to primary sclerosing cholangitis (PSC) but originates from a known pathological process. Its clinical and cholangiographic features may mimic PSC, yet its natural history may be more favorable if recognition is prompt and appropriate therapy is introduced. Sclerosing cholangitis in critically ill patients, however, is associated with rapid disease progression and poor outcome. Serologic testing, radiological imaging and histological analysis can help diagnose SSC.