Clinical History
Admitted to hospital with a hemorrhagic infarction of the left occipital lobe. He was a hypertensive and diabetic for the last 6 years. The routine tests revealed hepatic dysfunction and cholestasis.
Imaging Findings
The patient, hypertensive and diabetic for the last 6 years, was admitted to our hospital because of a hemorrhagic cerebral infarction. During his stay the routine biochemical and hematologic tests revealed hepatic dysfunction. He had elevated serum alkaline phosphatase and γGT. Serum bilirubin and the markers of liver function were in normal ranges.
Ultrasound demonstrated diffuse heterogenicity of the liver and no biliary obstruction. Further evaluation for a possible diffuse liver disease revealed elevated serum IgM concentration and antibodies against nuclear proteins.
CT of the upper abdomen showed a mild lobular contour of the liver, a relative enlargement of the left liver lobe and linear hypoattenuating parenchymal areas, findings suggestive of cirrhosis.
The patient underwent MRI of the liver which confirmed the presence of the above mentioned morphological changes. In addition, a lacelike pattern of diffuse fibrosis was shown on T2-weighted and contrast enhancement T1-weighted images. MRCP was normal (not shown).
The patient underwent percutaneous liver biopsy. Histological examination revealed a necrotizing inflammatory process involving the portal triads with destruction of small- and medium–sized bile ducts, development of a dense infiltrate of acute and chronic inflammatory cells, and mild fibrosis, establishing the diagnosis of primary biliary cirrhosis (PBC).
Discussion
Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease. It is characterized by inflammatory destruction of the small bile ducts within the liver. PBC eventually leads to cirrhosis of the liver and is the third most common indication for liver transplantation in adults. No known causes for PBC have been identified. The typical patient is a middle-aged woman presenting with symptoms of fatigue and pruritus and laboratory test evidence of cholestasis.
The presence of intra- and extrahepatic imaging findings of cirrhosis (eg, surface nodularity, hepatic morphologic changes, splenomegaly, ascites, portosystemic collaterals) depends on the severity of the underlying chronic liver disorder. Enlargment of the hilar periportal space, in the absence of other abnormalities, has been described as a helpful sign in the diagnosis of early cirrhosis. More pronounced lobar or segmental changes of hepatic morphology are seen in advanced cirrhosis.
In patients with end-stage cirrhosis the liver usually appears diffusely atrophic or shows a combination of segmental atrophy and hypertrophy. Patients with PBC have almost exclusively a diffuse hypertrophic liver. Most forms of diffuse fibrosis can occur in any type of cirrhosis. In PBC a high prevalence of thin perilobular bands has been reported, giving the appearance of lacelike form as it was shown in our patients.
Abdominal lympadenopathy is seen in 86% of the patients with PBC compared with 37%-49% of patients with other forms of cirrhosis.
Recently, a radiologic sign, that was found specific for PBC has been described: a low signal-intensity abnormality centered around portal venous branches on contrast enhanced T1 and T2-weighted MR images (periportal halo sign), distinct from regenerating nodules. This sign has been attributed to the deposition of fibrous tissue or cellular depletion around the portal triads.
In conclusion we presented a patient with intrahepatic cholestasis and imaging findings of early cirrhosis with lacelike fibrosis. Elevated serum IgM concentration arose the suspicion and the liver biopsy established the diagnosis of PBC.
Differential Diagnosis List
Primary biliary cihrrosis (PBC).
Final Diagnosis
Primary biliary cihrrosis (PBC).
