Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Dec;8(1):6.
doi: 10.1007/s13317-017-0094-5. Epub 2017 Apr 28.

A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage

Moris Sangineto  1 , Chiara Valentina Luglio  2 , Patrizia Suppressa  2 , Carlo Sabbà  2 , Nicola Napoli  2
Affiliations

A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage

Moris Sangineto et al. Auto Immun Highlights. 2017 Dec.

Abstract

Sarcoidosis is a systemic disease characterized by an immune-mediated disorder, which leads to the development of non-caseating granulomas in the involved organs. More than 90% of patients with sarcoidosis present lungs and lymphatic system involvement at onset, while less than 10% has an isolated extrapulmonary localization. Here, we describe the case of an elderly patient with isolated hepato-splenic onset (multiple splenic lesions at imaging and cholestasis), and subsequent pulmonary involvement. The liver biopsy showed the presence of non-caseating granulomas, suggesting sarcoidosis. Despite the complete recovery was obtained with steroid therapy, after dosage reduction the patient presented watery diarrhea. Endoscopic investigations with biopsies were performed, describing the presence of an important lympho-plasmacytic infiltrate of terminal ileum mucosa with typical aspects of inflammatory bowel disease. The symptomatology completely disappeared after steroid dosage increase. This case confirms that sarcoidosis could present in a very atypical way, involving several organs in a different manner at the same time and that every symptom should not be underestimated, despite the rare presentation.

Keywords: Crohn’s disease; Intestinal sarcoidosis; Liver; Sarcoidosis; Spleen.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest

None.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent and animal rights

There is not an informed consent because this article does not contain any experimentation with human subjects, neither identifying elements.

Figures

Fig. 1
Fig. 1
The magnetic resonance imaging shows multiple splenic lesions (a) and reduction of caliber of the proximal extrahepatic bile duct with ectasia of bile duct downstream (b) at disease onset. One month later, a new total body computerized tomography describes a dense tissue (diameter of 3 cm) with irregular margins infiltrating the upper lobe of the left lung, associated with ipsilateral hilar-mediastinal lymphadenopathy (c)
Fig. 2
Fig. 2
a Hematoxylin and eosin staining of liver tissue, showing a sarcoid, non-caseating granuloma, with the presence of typical epithelioid and giant cells (magnification ×20). b–d Hematoxylin and eosin staining of ileum mucosa, showing a lymphoplasmacytic and granulocyte infiltrate, with erosions, emperipolesis, glandular atrophy and distortion, and nodular lymphoid aggregates in the ileum mucosa (magnification ×2, ×10 and ×10, respectively)
Fig. 3
Fig. 3
The computerized tomography shows a complete resolution of the splenic lesions (a) and of the lung infiltration (b)
See this image and copyright information in PMC

References

    1. Nunes H, Bouvry D, Soler P, Valeyre D. Sarcoidosis. Orphanet J Rare Dis. 2007;2:46. doi: 10.1186/1750-1172-2-46. - DOI - PMC - PubMed
    1. Baughman RP, Lower EE, du Bois RM. Sarcoidosis. Lancet. 2003;361:1111–1118. doi: 10.1016/S0140-6736(03)12888-7. - DOI - PubMed
    1. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999 (1999) Am J Respir Crit Care Med 160:736–755 - PubMed
    1. Giovinale M, Fonnesu C, Soriano A, Cerquaglia C, Curigliano V, Verrecchia E, De SG, Gasbarrini G, Manna R. Atypical sarcoidosis: case reports and review of the literature. Eur Rev Med Pharmacol Sci. 2009;13(Suppl 1):37–44. - PubMed
    1. Vatti R, Sharma OP. Course of asymptomatic liver involvement in sarcoidosis: role of therapy in selected cases. Sarcoidosis Vasc Diffuse Lung Dis. 1997;14:73–76. - PubMed

LinkOut - more resources