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

southeast Asia Southeast Asia, also spelled South East Asia and South-East Asia, and also known as Southeastern Asia, South-eastern Asia or SEA, is the geographical United Nations geoscheme for Asia#South-eastern Asia, south-eastern region of Asia, consistin ...

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 hormones. There are two main types: acute pancreatitis, and chronic p ...

, 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 its junction with the duodenum (first part of the small intestine). It tends to occur if ...

, gallbladder

empyema An empyema () is a collection or gathering of pus within a naturally existing anatomical cavity. For example, pleural empyema is empyema of the pleural cavity. It must be differentiated from an abscess, which is a collection of pus in a newly for ...

, clotting within the

hepatic The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...

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. App ...

sepsis Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is foll ...

and death. Chronic biliary obstruction may cause

jaundice Jaundice, also known as icterus, is a yellowish or 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 abnormal heme met ...

itchiness Itch (also known as pruritus) is a sensation that causes the desire or reflex to scratch. Itch has resisted many attempts to be classified as any one type of sensory experience. Itch has many similarities to pain, and while both are unpleasant s ...

, liver abscesses, and

cirrhosis Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue repai ...

, particularly at the left lobe segment 3, and can eventually lead to

intraductal papillary mucinous neoplasm Intraductal papillary mucinous neoplasm (IPMN) is a type of tumor that can occur within the cells of the pancreatic duct. IPMN tumors produce mucus, and this mucus can form pancreatic cysts. Although intraductal papillary mucinous neoplasms are ...

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 one of the world's most notable geographical regions, which is either considered a continent in its own right or a subcontinent of Eurasia, which shares the continental landmass of Afro-Eurasia with Africa. Asia covers an ...

and very rare in

Europe Europe is a large peninsula conventionally considered a continent in its own right because of its great physical size and the weight of its history and traditions. Europe is also considered a subcontinent of Eurasia and it is located enti ...

and the

United States The United States of America (U.S.A. or USA), commonly known as the United States (U.S. or US) or America, is a country primarily located in North America. It consists of 50 U.S. state, states, a Washington, D.C., federal district, five ma ...

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

bile Bile (from Latin ''bilis''), or gall, is a dark-green-to-yellowish-brown fluid produced by the liver of most vertebrates that aids the digestion of lipids in the small intestine. In humans, bile is produced continuously by the liver (liver b ...

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

cholesterol Cholesterol is any of a class of certain organic molecules called lipids. It is a sterol (or modified steroid), a type of lipid. Cholesterol is biosynthesized by all animal cells and is an essential structural component of animal cell membr ...

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. also known as ''E. coli'' (), is a Gram-negative, facultative anaerobic, rod-shaped, coliform bacterium of the genus '' Esc ...

'' 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 Trematoda, phylum Platyhelminthes. It infects fish-eating mammals, including humans. In humans, it infects the common bile duct and gall bladder, feeding ...

and

Ascaris lumbricoides ''Ascaris lumbricoides'' is a large parasitic worm that causes ascariasis in humans. A roundworm of genus '' Ascaris'', it is the most common parasitic worm in humans. An estimated one-sixth of the human population is at some point infected by a ...

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 organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...

, typically with CT,

ultrasound Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies fr ...

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

. 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 ( or faeces), known colloquially and in slang as poo and poop, 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 large intestine. Feces contain a relati ...

or in

duodenal The duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear, and the terms anterior intestine or proximal intestine m ...

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 uses a solid-phase type of enzyme immunoassay (EIA) to detect the presence ...

, which has become the most important clinical technique. Diagnosis by detecting DNAs from eggs in faeces are also developed using

PCR PCR or pcr may refer to: Science * Phosphocreatine, a phosphorylated creatine molecule * Principal component regression, a statistical technique Medicine * Polymerase chain reaction ** COVID-19 testing, often performed using the polymerase chain r ...

, real-time PCR, and

LAMP Lamp, Lamps or LAMP may refer to: Lighting * Oil lamp, using an oil-based fuel source * Kerosene lamp, using kerosene as a fuel * Electric lamp, or light bulb, a replaceable component that produces light from electricity * Light fixture, or lig ...

, 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 partial resections of hepatic tissue and hepat ...

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

References

External links