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Published on: February 17, 2018
A rare course of Whipple's disease with atypical cardiac manifestation
S Gunia1, M H Behrens, P Stosiek
1Institut für Pathologie, Carl-Thiem-Klinikum, Cottbus, Germany. S.Gunia@ctk.de
Abstract:
Whipple's disease (WD) is a rare disorder caused by the gram-positive actinomycete Tropheryma whippelii (1). An oral route of acquisition is presumed (2). WD is a chronic systemic infection morphologically characterized by foamy PAS-positive macrophages. This case report depicts the diagnosis and clinical management of a woman who presented with atypical cardial manifestations of WD and died from severe cardiac impairment by Tropheryma whippelii which was confirmed by polymerase chain reaction. Histological assessment of myocardial specimens obtained at autopsy showed PAS-positive macrophages accumulating within foci of interstitial myocardial fibrosis. Immunohistochemical staining demonstrated myocarditis with lymphocytes and neutrophilic granulocytes, which has not been reported so far in the course of WD.
Insights
Whipple's disease, a rare infection, can cause severe cardiac issues. This case highlights Tropheryma whippelii causing myocarditis and cardiac death, a previously unreported manifestation.
Area of Science:
- Infectious Diseases
- Cardiology
- Pathology
Background:
- Whipple's disease (WD) is a rare chronic systemic infection caused by Tropheryma whippelii.
- The oral route is the presumed acquisition pathway for WD.
- WD is characterized by foamy, PAS-positive macrophages in affected tissues.
Observation:
- A case report details a woman with atypical cardiac manifestations of WD.
- The patient experienced severe cardiac impairment due to Tropheryma whippelii infection.
- Autopsy revealed PAS-positive macrophages in myocardial fibrotic foci.
Findings:
- Polymerase chain reaction confirmed Tropheryma whippelii in myocardial tissue.
- Histological analysis showed interstitial myocardial fibrosis.
- Immunohistochemistry revealed myocarditis with lymphocytes and neutrophilic granulocytes, a novel finding in WD.
Implications:
- This case expands the understanding of Whipple's disease clinical presentations.
- It underscores the potential for Tropheryma whippelii to cause severe, previously unreported cardiac pathology.
- Highlights the importance of considering WD in cardiac impairment cases, even with atypical symptoms.
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