Musculoskeletal ImagingBiliary cirrhosis
a rare disorder of the liver of unknown cause characterized by destruction of small intrahepatic bile ducts with sparing of major intra- and extrahepatic ducts. Women are affected almost exclusively. The presence of antimitochondrial antibodies and characteristic findings on liver
biopsy confirm the diagnosis. For further general description, see
biliary cirrhosis.
Patients exhibit osteomalacia, osteoporosis, arthralgias or arthritis, and hypertrophic osteoarthropathy; coexisting connective tissue diseases, such as scleroderma or Sjogren's syndrome, may also be present. Xanthomas may form in soft tissues and in subperiosteal and intramedullary locations as a consequence of hypercholesterolaemia. An erosive arthritis of the hands and wrists may sometimes be seen in patients with primary biliary cirrhosis. Some patients also exhibit a destructive arthropathy of large joints.
Radiographic manifestations of biliary cirrhosis in the peripheral joints include soft tissue swelling, joint space narrowing, erosions, joint destruction and rarely chondrocalcinosis.
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