A case of ²sarcoidosis like² Whipple‘s disease

S. Lachkar, Y. Benhamou, F. Corne, H. Levesque, L. Thiberville (Rouen, France)

Source: Annual Congress 2006 - Sarcoidosis
Session: Sarcoidosis
Session type: Electronic Poster Discussion
Number: 3136
Disease area: Interstitial lung diseases, Thoracic oncology

Congress or journal article abstract

Abstract

A 56 year old man without medical history presented to the hospital in August 2003 with polyarthralgias, a truncal maculo-papular rash and axillary lymphnodes. Laboratory data suggested a mild inflammatory state (CRP of 28mg/L and VS of 40mn) and an elevated Angiotensin Converting Enzyme at 50IU/L. Thorax CT scan displayed enlarged mediastinal lymphnodes. A Skin and lymph node biopsy showed a non-caseating granulomatosis suggesting the diagnosis of sarcoidosis. A treatment by hydroxychloroquine was started.
In June 2005 a third degree atrio-ventricular block occured, leading to a pacemaker placement. A transthoracic echocardiogram demonstrated a dilated cardiomyopathy with a left venticular ejection fraction of 30% . Nuclear imaging of the myocardium was compatible with sarcoid myocarditis. Corticosteroids and Methotrexate were initiated.
Seven weeks later, an esophageo-gastroduodenoscopy, demonstrated an ulcerated duodeno-jejunitis. Duodenal biopsies showed a histiocytic infiltrate and a positive PAS stain. PCR assay for Tropheryma whippelii was positive leading to the diagnosis of Whipple‘s disease. PCR was also positive on the 2003 skin biopsies and lymph nodes.
This case report demonstrates the similarities in clinical presentation between systemic sarcoidosis and Whipple‘s disease and the critical value of specific Tropheryma whippelii PCR assay, that can be performed from archived paraffin pathologic blocks. In our knowledge, this is also the second published case of a Whipple disease complicated by a myocarditis.


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S. Lachkar, Y. Benhamou, F. Corne, H. Levesque, L. Thiberville (Rouen, France). A case of ²sarcoidosis like² Whipple‘s disease. Eur Respir J 2006; 28: Suppl. 50, 3136

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