Hepatic and Splenic Sarcoidosis Without Lymphadenopathy: An Atypical Multisystem Presentation

TL;DR

A rare case of sarcoidosis affecting the liver and spleen without lymph node involvement is presented, highlighting diagnostic challenges.

Contribution

This case report adds to the understanding of atypical sarcoidosis presentations without lymphadenopathy.

Findings

A 66-year-old woman presented with chronic hepatosplenomegaly and cholestatic enzymes without lymphadenopathy.

Liver biopsy confirmed non-necrotizing granulomas consistent with hepatic sarcoidosis.

The case emphasizes the need to consider sarcoidosis in atypical multisystem presentations.

Abstract

Sarcoidosis is a multisystem granulomatous disease of unknown etiology characterized by non-caseating granulomas in affected organs. Although pulmonary involvement is most common, extrapulmonary manifestations, particularly hepatic and splenic, may occur in isolation or association. The absence of lymphadenopathy is considered an atypical and uncommon pattern, which may hinder the diagnosis and requires the exclusion of other causes of granulomatous disease. We present a case of a 66-year-old woman with chronic hepatosplenomegaly, mild cytopenias, persistent elevation of cholestatic enzymes, and progressive pulmonary changes without lymphadenopathy. Extensive investigation excluded infectious, autoimmune, and hematologic causes. Liver biopsy confirmed non-necrotizing granulomas consistent with hepatic sarcoidosis. This case highlights the diagnostic challenges posed by atypical sarcoidosis presentations and underscores the importance of considering sarcoidosis in the differential diagnosis of isolated hepatosplenomegaly, even in the absence of lymphadenopathy.

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