Timothy C. Hain, MD Return to Index. Page last modified: February 15, 2017
Whipple's disease is a rare chronic multi-systemic infectious disorder caused by the Gram-positive bacillus, Tropheryma whippelii. It may affect any organ in the body, but most commonly it presents with gastrointestinal problems. (Maigra et al, 2005)
According to Deriban and Marth (2006), a defect in cellular immune response may predispose patients for an infection with T. whipplei and this might explain the rarity of the disorder despite the ubiquitous bacterial presence. The presumed immunological defect is likely to be quite specific for T. whipplei, since patients are not generally affected by other infections. Decreased production of Interleukin(IL)-12, IL-2 and Interferon (IFN)-g accompanied by an increased secretion of IL-4 are the main features of this defective immunological response.
The nystagmus of Whipples consists of a "convergent-divergent pendular nystagmus associated with a synchronous, rhythmic movement of the mouth, jaw, and extremities. " (Simpson et al, 1995)
In typical Whipple's disease, the most severe changes are seen in the proximal small intestine and biopsy reveals mucosal and lymph node infiltration with large, foamy histocytes, containing granules that stain positive with periodic acid-Schiff (PAS) reagent and represent intact or partially degraded bacteria.
PCR for Whipples (best done on intestinal tissue) is more sensitive than biopsy (Lehmann et al, 2016; Kono et al, 2015). Some authors report that serum PCR for Whipples is helpful (Renon et al, 2012).
Occasionally, Serum and CSF PCR for T whipplei is negative and biopsy is the only positive test (Mohamed et al, 2011).
Extended antibiotic treatment (up to 1-year) is indicated. Life-long surveillance for recurrence is essential, once primary treatment has been completed (Maigra et al, 2005) . Untreated WD patients suffer from a chronic progressive disorder which possibly leads to death. Most patients show a fast clinical improvement to antibiotic therapy, but clinical relapses are described frequently. There is a number of patients, unable to eradicate the bacterium even after several antibiotic treatments and patients with CNS disease, in both of whom alternative therapy strategies are necessary. (Deriban and Marth, 2006)
According to Simpson et al (1995), current treatment consists of intravenous trimethoprim-sulfamethoxazole for 2 weeks followed by oral trimethoprim-sulfamethoxazole twice daily for 1 year.
Cephtriaxone is reported by some to be useful, as well (Renon et al, 2012).