title: Hepatic sarcoidosis in patients presenting with liver dysfunction: imaging appearance, pathological correlation and disease evolution.
abstract: OBJECTIVE
  We hypothesize that hepatic sarcoidosis is a dynamic process that can lead to cirrhosis and portal hypertension, independent of the course of thoracic disease. Therefore, we assess the imaging appearance and progression of hepatic sarcoidosis in subjects presenting with hepatic dysfunction.
  
  
  METHODS
  An IRB-approved, HIPAA-compliant, single-institution retrospective review identified 39 subjects with sarcoidosis-related liver dysfunction. Clinical information was collected. Two abdominal radiologists analyzed baseline and follow-up imaging studies, scoring features of cirrhosis. Chest CT was also analyzed.
  
  
  RESULTS
  At presentation, 23 subjects (59.0 %) exhibited >3 cirrhotic features and 15 (38.5 %) >2 findings of portal hypertension. Of subjects with available follow-up, 57.9 % (19 subjects; mean interval 4.7 years) showed worsening of >3 cirrhotic features (Pearson rho = 0.58; p = 0.009). Parenchymal nodules were uncommon (25.6 %), and most regressed. Although 87.2 % of subjects were diagnosed with thoracic sarcoidosis, there was poor correlation between severity of hepatic and chest disease (Pearson rho = 0.30; p = 0.119). A mean of 7.2 years elapsed between diagnosis of pulmonary and liver involvement.
  
  
  CONCLUSIONS
  Sarcoidosis may present as liver dysfunction, cirrhosis or portal hypertension. Sarcoid-related liver disease may progress and can manifest without, alongside or significantly after a diagnosis of pulmonary disease.
  
  
  CONCLUSIONS
  • Patients often present with elevated liver function tests indicating cholestasis. • Patients may present with portal hypertension, and some progress to cirrhosis. • Though biopsy can be considered for focal liver lesions, most will regress. • Extent of intra-abdominal involvement may not correlate with severity of thoracic disease. • Liver disease may manifest alongside, prior to or significantly after initial diagnosis.
authors.affiliation: Department of Radiology, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390-8896, USA. david.fetzer@utsouthwestern.edu.
authors.forename: David T
authors.identifier: http://orcid.org/0000-0002-2420-082X
authors.initials: DT
authors.lastname: Fetzer
authors.affiliation: Department of Radiology, University of Pittsburgh, 200 Lothrop St, Pittsburgh, PA, 15213, USA.
authors.forename: Mitchell A
authors.initials: MA
authors.lastname: Rees
authors.affiliation: Department of Radiology, University of Pittsburgh, 200 Lothrop St, Pittsburgh, PA, 15213, USA.
authors.forename: Anil K
authors.initials: AK
authors.lastname: Dasyam
authors.affiliation: Department of Radiology, University of Pittsburgh, 200 Lothrop St, Pittsburgh, PA, 15213, USA.
authors.forename: Mitchell E
authors.initials: ME
authors.lastname: Tublin
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000473:pathology; D008099:Liver / Q000000981:diagnostic imaging*; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D061330:Multidetector Computed Tomography / Q000379:methods*; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis*
keywords: Chronic hepatitis; Cirrhosis; Liver diseases; Portal hypertension; Sarcoidosis complications