A 51 year old male with long standing alcohol abuse and presumed cirrhosis presented with a 6 month history of worsening right heart failure symptoms, a 1 year history of diarrhea, weight loss, polyarthralgias, low grade fever, and generalized lymphadenopathy.
Physical examination revealed a diastolic heart murmur and basal rales. Echocardiography confirmed Aortic Insufficiency (AI) with chronic vegetation on the valve. Multiple blood cultures, serologies, in addition to Tropheryma whipplei (T. whipplei) polymerase chain reaction (PCR) assay in blood, were negative. Inguinal node biopsy was unrevealing. He was referred for cardiac surgery due to heart failure refractory to medical therapy. As part of his preoperative evaluation, a liver biopsy was obtained to clarify his presumed diagnosis of cirrhosis. Biopsy showed no evidence of cirrhosis, but cells with periodic acid‐Schiff (PAS) stain‐positive macrophages were seen. However no organisms were identified and PCR for T. whippei was negative. He subsequently underwent an aortic valve replacement. Pathology of the valve showed foamy macrophages with PAS positive microorganisms, again with a negative PCR. Ceftriaxone was initiated for a 6 week course. The patient had a marked clinical improvement of his arthralgias and digestive symptoms on antibiotic therapy, and remained asymptomatic from a cardiac standpoint. He was placed on long‐term trimethoprim‐sulfamethoxazole.
Whipple’s disease is rare (<1 case per 1 million people per year) and is caused by the bacterium T. whipplei. This chronic multi‐system disease often presents with a triad of diarrhea, weight loss, and malabsortion. Arthralgias are common. Generalized lymphadenopathy and neurologic complaints have been reported. Whipple’s Disease presents with cardiac manifestations in 20‐55% of patients, frequently involving the aortic valve. Whipple’s endocarditis has rarely been known to occur in patients without systemic Whipple’s disease.
Diagnosis involves a combination of pathology and PCR analysis. The combination of PAS stain, PCR confirmation with sequencing, and/or immunohistochemistry using antibody specific against T. whippleishould be employed as misidentification is possible due to contamination of sample or PCR reagents with unrelated bacterial DNA, or lack of sequence divergence between species. Treatment of Whipple’s endocarditis involves both surgical and medical (antimicrobial) therapy.
Culture negative endocarditis is often the result of prior antimicrobial therapy. However, hospitalists should have Whipple’s Disease as a differential diagnosis in patients with culture negative endocarditis, especially when a patient presents with chronic diarrhea, weight loss, arthralgia, and lymphadenopathy. Greater awareness of the possibility of this disease as a cause of AI and endocarditis is paramount, because in addition to surgery, prolonged antibiotic course is mandatory. Without treatment, Whipple’s Disease is ultimately fatal.
To cite this abstract:Barbastefano J, Mathias J, Barbastefano N, Fraser T. Whipple’s Endocarditis– an Elusive Diagnosis. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 340. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/whipples-endocarditis-an-elusive-diagnosis/. Accessed March 28, 2020.