Whipple's disease: a rare disease revisited.
Since the original postmortem diagnosis of "intestinal lipodystrophy" by Dr. George H. Whipple in 1907, the complexities of Whipple's disease have been elucidated through case reports. Universally fatal prior to the advent of antibiotics, Tropheryma whipplei is increasingly recognized as an organism that can be treated only if the clinician seeks to identify it. Whipple's disease is primarily a gastrointestinal disease manifesting as a malabsorption syndrome, and is detected through endoscopy and intestinal biopsy. Nongastrointestinal manifestations of the disease, although less common, are reported and have aided in its recognition as a multiorgan disease entity. Because of its rarity, treatment recommendations are currently based on observational studies and on one recent prospective study, which outlined induction therapy followed by several months of suppressive maintenance therapy to prevent relapse, which is often characterized by neurologic symptoms.
Department of Gastroenterology/Hepatology, Scripps Clinic, 10666 North Torrey Pines Road, La Jolla, CA 92037, USA. afshar.payam@scrippshealth.org
Payam
P
Afshar
David C
DC
Redfield
Philip A
PA
Higginbottom
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002908:Chronic Disease; D006801:Humans; D016133:Polymerase Chain Reaction; D035583:Rare Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000382:microbiology; D016896:Treatment Outcome; D054851:Tropheryma / Q000235:genetics / Q000472:pathogenicity*; D008061:Whipple Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000382:microbiology
D000900:Anti-Bacterial Agents