Recurrent pyogenic cholangitis (RPC), also known as Hong Kong disease, Oriental cholangitis, and Oriental infestational cholangitis, is a chronic infection characterized by recurrent bouts of bacterial cholangitis with primary

hepatolithiasis Hepatolithiasis is the presence of gallstones in the biliary ducts of the liver. Treatment is usually surgical. It is rare in Western countries, but prevalent in East Asia.* The gallstones are normally found proximal to the left and right hepati ...

. It is exclusive to people who live or have lived in

southeast Asia Southeast Asia is the geographical United Nations geoscheme for Asia#South-eastern Asia, southeastern region of Asia, consisting of the regions that are situated south of China, east of the Indian subcontinent, and northwest of the Mainland Au ...

Presentation

Presentation can be atypical and without pain or fever, especially in the elderly. Positive symptoms include

biliary colic Biliary colic, also known as symptomatic cholelithiasis, a gallbladder attack or gallstone attack, is when a colic (sudden pain) occurs due to a gallstone temporarily blocking the cystic duct. Typically, the pain is in the right upper part of t ...

, acute

pancreatitis Pancreatitis is a condition characterized by inflammation of the pancreas. The pancreas is a large organ behind the stomach that produces digestive enzymes and a number of hormone A hormone (from the Ancient Greek, Greek participle , "se ...

, obstructive jaundice and less commonly, liver enlargement and abnormal liver function tests. Additional complications in the acute setting include

ascending cholangitis Ascending cholangitis, also known as acute cholangitis or simply cholangitis, is inflammation of the bile duct, usually caused by bacteria ascending from Ampulla of Vater, its junction with the duodenum (first part of the small intestine). It ten ...

, gallbladder

empyema An empyema (; ) is a collection or gathering of pus within a naturally existing anatomical cavity. The term is most commonly used to refer to pleural empyema, which is empyema of the pleural cavity. It is similar or the same in meaning as an a ...

, clotting within the

hepatic The liver is a major metabolic organ (anatomy), organ exclusively found in vertebrates, which performs many essential biological Function (biology), functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of var ...

and

portal vein The portal vein or hepatic portal vein (HPV) is a blood vessel that carries blood from the gastrointestinal tract, gallbladder, pancreas and spleen to the liver. This blood contains nutrients and toxins extracted from digested contents. Approxima ...

sepsis Sepsis is a potentially life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage of sepsis is followed by suppression of the immune system. Common signs and s ...

and death. Chronic biliary obstruction may cause

jaundice Jaundice, also known as icterus, is a yellowish or, less frequently, greenish pigmentation of the skin and sclera due to high bilirubin levels. Jaundice in adults is typically a sign indicating the presence of underlying diseases involving ...

itchiness An itch (also known as pruritus) is a sensation that causes a strong desire or reflex to scratch. Itches have resisted many attempts to be classified as any one type of sensory experience. Itches have many similarities to pain, and while both ...

, liver

abscess An abscess is a collection of pus that has built up within the tissue of the body, usually caused by bacterial infection. Signs and symptoms of abscesses include redness, pain, warmth, and swelling. The swelling may feel fluid-filled when pre ...

es, and

cirrhosis Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, chronic liver failure or chronic hepatic failure and end-stage liver disease, is a chronic condition of the liver in which the normal functioning tissue, or parenchyma, is replaced ...

, particularly at the left lobe segment 3, and can eventually lead to intraductal papillary mucinous neoplasm or

cholangiocarcinoma Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the bile ducts. Symptoms of cholangiocarcinoma may include abdominal pain, yellowish skin, weight loss, generalized itching, and fever. Light colored stoo ...

Pathogenesis

With RPC, the gallstones found within the biliary system are made of calcium bilirubinate or pigmented calcium. Calcium bilirubinate stones are prevalent in

Asia Asia ( , ) is the largest continent in the world by both land area and population. It covers an area of more than 44 million square kilometres, about 30% of Earth's total land area and 8% of Earth's total surface area. The continent, which ...

and very rare in

Europe Europe is a continent located entirely in the Northern Hemisphere and mostly in the Eastern Hemisphere. It is bordered by the Arctic Ocean to the north, the Atlantic Ocean to the west, the Mediterranean Sea to the south, and Asia to the east ...

and the

United States The United States of America (USA), also known as the United States (U.S.) or America, is a country primarily located in North America. It is a federal republic of 50 U.S. state, states and a federal capital district, Washington, D.C. The 48 ...

. They tend to be friable concretions of various shapes and sizes within the biliary tree, and their associated

bile Bile (from Latin ''bilis''), also known as gall, is a yellow-green/misty green fluid produced by the liver of most vertebrates that aids the digestion of lipids in the small intestine. In humans, bile is primarily composed of water, is pro ...

is often muddy in consistency and contains numerous fine particles of calcium bilirubinate as well. This differs greatly from

cholesterol Cholesterol is the principal sterol of all higher animals, distributed in body Tissue (biology), tissues, especially the brain and spinal cord, and in Animal fat, animal fats and oils. Cholesterol is biosynthesis, biosynthesized by all anima ...

stones, which are common in Europe and the United States. The formation of calcium bilirubinate stones in RPC has been attributed to a high incidence of infection with ''

Escherichia coli ''Escherichia coli'' ( )Wells, J. C. (2000) Longman Pronunciation Dictionary. Harlow ngland Pearson Education Ltd. is a gram-negative, facultative anaerobic, rod-shaped, coliform bacterium of the genus '' Escherichia'' that is commonly fo ...

'' in bile. In humans, the majority of bilirubin is excreted in the bile as bilirubin glucuronide. Hepatolithiasis is associated with

Clonorchis sinensis ''Clonorchis sinensis'', the Chinese liver fluke, is a liver fluke belonging to the class trematode, Trematoda, phylum Platyhelminthes. It infects fish-eating mammals, including humans. In humans, it infects the common bile duct and gall bladder, ...

and

Ascaris lumbricoides ''Ascaris lumbricoides'' is a large parasitic worm, parasitic Nematoda, roundworm of the genus ''Ascaris.'' It is the most common parasitic worm in humans. An estimated 807 million–1.2 billion people are infected with ''Ascaris lumbricoides'' ...

infestation of the liver. This theory is based on high incidence of dead parasites or ova within stone in autopsy findings.

Diagnosis

The diagnosis can be suspected by imaging, with typical characteristics centering around appearance of the

liver The liver is a major metabolic organ (anatomy), organ exclusively found in vertebrates, which performs many essential biological Function (biology), functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of var ...

, typically with CT,

ultrasound Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...

. Traits that raise suspicion for the infection include intra- and extra- hepatic dilatation and strictures with intraductal pigmented stones, usually in the absence of gallstones and with regions of segmental liver atrophy, particularly the lateral aspect of the left hepatic lobe. There is also reduced arborization of peripheral ducts. Approximately 5% of chronic infections go on to develop

. Pathogenic bacteria responsible for the infection include ''E. coli'', ''Klebsiella'', ''Pseudomonas'', and ''Proteus'' species. Anaerobes are a less frequent cause, and a culture positive for multiple strains can be common. When related to

, definitive diagnosis is by identification of eggs by microscopic demonstration in

faeces Feces (also known as faeces American and British English spelling differences#ae and oe, or fæces; : faex) are the solid or semi-solid remains of food that was not digested in the small intestine, and has been broken down by bacteria in the ...

or in

duodenal The duodenum is the first section of the small intestine in most vertebrates, including mammals, reptiles, and birds. In mammals, it may be the principal site for iron absorption. The duodenum precedes the jejunum and ileum and is the shortest p ...

aspirate, but other sophisticated methods have been developed, such as

ELISA The enzyme-linked immunosorbent assay (ELISA) (, ) is a commonly used analytical biochemistry assay, first described by Eva Engvall and Peter Perlmann in 1971. The assay is a solid-phase type of enzyme immunoassay (EIA) to detect the presence of ...

, which has become the most important clinical technique. Diagnosis by detecting DNAs from eggs in faeces are also developed using PCR, real-time PCR, and LAMP, which are highly sensitive and specific.

Treatment

The treatment of RPC involves management of sepsis during episodes of cholangitis with antibiotics, abscess drainage, and blood pressure support. With resistant infection, a surgical

hepatectomy Hepatectomy is the surgical resection (removal of all or part) of the liver. While the term is often employed for the removal of the liver from a liver transplant donor, this article will focus on Segmental resection, partial resections of hepatic ...

or hepaticocutaneousjejunostomy can be performed. Lifelong surveillance for malignancy is also usually necessary.

Presentation

Pathogenesis

Diagnosis

Treatment

See also