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Case report
Extrapulmonary sarcoidosis primarily presenting as cholestatic liver disease
  1. Pieter De Mulder1,
  2. Bert Maertens1,
  3. Anne Hoorens2 and
  4. An Vonck3
  1. 1 Internal Medicine, Ghent University, Gent, Belgium
  2. 2 Anatomopathology, Ghent University, Gent, Belgium
  3. 3 Nephrology, Algemeen Ziekenhuis Sint Blasius campus Dendermonde, Dendermonde, Belgium
  1. Correspondence to Dr Pieter De Mulder; pieter.de.mulder{at}hotmail.com

Abstract

Sarcoidosis is a multisystem inflammatory disorder associated with non-caseating granulomas in affected organs, most commonly the lungs. Involvement of extrapulmonary organs is common, but lack of pulmonary involvement is rare and is called non-pulmonary sarcoidosis (NPS). Like pulmonary sarcoidosis, a definitive diagnostic test for NPS does not exist. Instead, the diagnosis of sarcoidosis requires the following elements: clinical and radiographic manifestations of sarcoidosis, histopathological detection of non-caseating granulomas and the exclusion of other diseases that may present similarly. Because of the experience with corticosteroids in pulmonary sarcoidosis, they are generally considered first-line therapy for NPS too. Ursodeoxycholic acid can be used to reduce cholestasis in NPS, but is inferior to corticosteroids in reducing inflammation. We hereby present a case that is particularly notable for its rare presentation of NPS as a granulomatous hepatitis with cholestatic liver function tests.

  • liver disease
  • hepatitis other
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Footnotes

  • PDM and BM are joint first authors.

  • Contributors PDM and BM are the joint first authors of this case report, responsible for the concept and design of the article. Both have been drafting the article and revising it critically for intellectual content. PDM wrote the summary, learning points, case presentation and investigations. BM wrote the background, the treatment section and the discussion section. The differential diagnosis was made by all four authors. Figures were made by AH. AV created the five tables. All the authors have approved the final admission and have ensured the accuracy and integrity of the article.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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