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Hepatic sarcoidosis in patients presenting with liver dysfunction: imaging appearance, pathological correlation and disease evolution

  • Hepatobiliary-Pancreas
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Abstract

Objectives

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.

Conclusion

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.

Key points

• 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.

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Abbreviations

ACE:

Angiotensin-converting enzyme

ALP:

Alkaline phosphatase

LFT:

Liver function test

GGT:

Gamma-glutamyl transferase

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Acknowledgments

The scientific guarantor of this publication is David T. Fetzer, MD. The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article. The authors state that this work has not received any funding. Takeshi Yokoo, MD, PhD, kindly provided statistical advice for this manuscript. Institutional Review Board approval was obtained. Written informed consent was waived by the Institutional Review Board. Methodology: retrospective, observational, performed at one institution.

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Authors and Affiliations

  1. Department of Radiology, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390-8896, USA

    David T. Fetzer

  2. Department of Radiology, University of Pittsburgh, 200 Lothrop St, Pittsburgh, PA, 15213, USA

    Mitchell A. Rees, Anil K. Dasyam & Mitchell E. Tublin

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  1. David T. Fetzer
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  2. Mitchell A. Rees
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Correspondence to David T. Fetzer.

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Fetzer, D.T., Rees, M.A., Dasyam, A.K. et al. Hepatic sarcoidosis in patients presenting with liver dysfunction: imaging appearance, pathological correlation and disease evolution. Eur Radiol 26, 3129–3137 (2016). https://doi.org/10.1007/s00330-015-4169-2

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  • DOI: https://doi.org/10.1007/s00330-015-4169-2

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