Snapshot A 60-year-old Caucasian man presents to the emergency room with his family for fever and recent onset of dementia. His MMSE score was 20 at his last appointment with his primary care provider. His past medical history includes chronic diarrhea of unknown origin and nonspecific arthritis. On physical exam, he has multiple hyperpigmented macules on his skin, as well as a new murmur. His physician is suspicious of endocarditis. Introduction Malabsorption syndrome with multi-organ involvement Caused by infection with Tropheryma whipplei login to view 4 more bullets Epidemiology More commonly in login to view 3 more bullets Risk factors login to view 2 more bullets Presentation Symptoms login to view 17 more bullets Physical exam login to view 6 more bullets Evaluation Endoscopic small bowel biopsy – most accurate test login to view 2 more bullets Stool PCR may reflect carrier state – not definitive CSF typically normal but should be performed Differential Diagnosis Celiac disease Crohn disease Lyme disease (arthralgias) Treatment Whipple without cardiac involvement login to view 2 more bullets Whipple with endocarditis login to view 2 more bullets Complications Jarisch-Herxheimer reaction to antibiotics login to view 3 more bullets Immune reconstitution inflammatory syndrome (IRIS) – up to 10% login to view 2 more bullets Prognosis Persistence after treatment common 30-50% rate of relapse Fatal if antibiotics not given