Primary intrahepatic lithiasis
Synonyms: PIHL | Primary hepatolithiasis
A rare biliary tract disease characterized by stone formation within the intrahepatic bile ducts without any known cause leading to bile stasis and repeated cholangitic episodes. The condition is rare in the Western world but frequent in eastern Asia. Patients usually present before the age of forty with right upper quadrant pain jaundice and/or fever. Stones are typically calcium bilirubinate (pigment) stones and bacteria are present in the bile in almost all cases. Complications are biliary strictures liver abscess liver fibrosis and secondary biliary cirrhosis. Association with cholangiocarcinoma has also been reported.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
Newly diagnosed with
Primary intrahepatic lithiasis?
Our RARE Concierge Services Guides are available to assist you by providing information, resources and connections as you navigate your rare disease journey.
Advocacy Organizations
Global Liver Institute
To improve the lives of individuals and families impacted by liver disease through promoting innovation, encouraging collaboration, and scaling optimal approaches to help eradicate liver diseases.
Clinical Trials
For a list of clinical trials in this disease area, please click here.