Treatment refractory arthritis and stroke - A case of infective endocarditis caused by Tropheryma whipplei

Abstract

Whipple´s disease is a rare multisystem condition affecting < 1/1.000.000 per year. The condition often presents with polyarthritis, diarrhea, and intestinal malabsorption. Endocarditis is seen in a minority of these patients, and is typically culture negative, as the causative agent Tropheryma whipplei does not grow in ordinary culture media. We present the case of a 78-year-old man with a history of seronegative polyarthritis that was refractory to treatment with several biological agents for a duration of 5 years prior to presentation to the emergency department with stroke. Echocardiography revealed aortic valve endocarditis with a 3.6 cm vegetation and multiple smaller vegetations. The patient underwent surgery with aortic valve replacement followed by prolonged antibiotic treatment. 16 S rDNA PCR analysis of the resected valve revealed T. whipplei as the causative agent. Two years after surgery and treatment with antibiotics, the patient's previously longstanding arthritis had totally disappeared and all rheumatological treatment had been discontinued.

Keywords: Culture-negative infective endocarditis; Immunomodulatory agents; Seronegative arthritis; Whipple’s disease.

Fig. 1

(A-E): Echocardiography in transthoracic 3 chambers view (A) and transesophageal long axis view (B) demonstrating a 3.6 cm long vegetation protruding into the left ventricular outflow tract during diastole. TEE showed a moderate aortic regurgitation (C). Transthoracic echocardiographic clip with 3 chamber view of the infected aortic valve (D). Transesophageal echocardiographic clip presenting a long axis view of the infected aortic valve (E).

Fig. 2

A; Picture of excised valve in the operating theater with involvement of all three cusps. B; Macroscopic image of the excised aortic valve after formalin fixation showing dimensions of the largest vegetation on the right aortic cusp (scale bar in centimeters).

Fig. 3

Histology (A-D) shows devitalized tissue with central areas with extensive calcifications (B) and peripheral areas with appearance of bacterial colonies (C-D). Hematoxylin-eosin (A-C) or gram staining, suboptimal staining quality due to devitalized tissue (D). Scale bar is 2 mm (A) or 0.2 mm (B-D).