Reviewed By: Pramod Kerkar, MD, FFARCSI

Whipple's disease (WD) is a chronic infection that affects the digestive tract, preferably the small intestine, joints, lymph nodes, heart, central nervous system, lungs and other tissues.

GH Whipple, in 1907, described the first case in a missionary physician. He named this entity intestinal lipodystrophy when finding numerous fatty deposits in the wall of the intestine and in the mesentery ganglia. The identification of the causal germ was done by genetic engineering in 1992. Since then it is known as Tropheryma whippelii and is included within the group actinomyces.

The Whipple's disease is unusual. The number of cases published in the last 90 years does not reach 2000.

What Is The Prognosis For Whipple's Disease?

When the treatment is effective, the prognosis for Whipple's disease is excellent. The Whipple's disease symptoms disappear in a month and remain asymptomatic.

Recurrence may be manifested as gastrointestinal symptoms or as extraintestinal symptoms, especially of the central nervous system (CNS) if the duration has not been correct (access of drugs to the CNS is not always good, although, for example, trimethoprim-sulfamethoxazole acts effectively in that location). The treatment of recurrences is more complex than the initial treatment. When the reintroduction of the initial treatment fails, other drugs can be tested, such as ceftriaxone, cefixime, rifampicin, and the combination of trimethoprim-sulfamethoxazole with recombinant-interferon-g.

It is expected that with the arrival of new diagnostic methods for Whipple's disease, such as serology, we will witness a notable increase in Whipple's disease, which will help to improve the epidemiological knowledge of this disease.

Treatment for Whipple's Disease

Since Whipple's disease was a bacterial disease with multisystem involvement, before the use of antibiotics it had an unfavorable prognosis.

The recommended treatment for Whipple's disease consists of intramuscular administration of 1.2 MU of benzylpenicillin (penicillin G) associated with 1 g of streptomycin daily for two weeks. This treatment should be continued by the oral administration of cotrimoxazole (trimethoprim 160 mg and sulfamethoxazole 800 mg) every 12 hours for a long period of time.

Once the treatment for Whipple's disease is established, the lesions usually resolve within a year, with 90% of the patients having a favorable evolution. Relapses are closely related to the duration of treatment. Thus, those who have completed less than six months have a higher recurrence rate, something that is not observed when the treatment has been performed for a year or more. Recurrences, which arise from cardiac and central nervous system localization, usually appear (on average) in the first four years (range between 2 months and 20 years). For this reason, some authors recommend its use indefinitely. Regardless of the duration of treatment, patients should be followed for a minimum of ten years.

The effectiveness of the Whipple's disease treatment can be monitored by polymerase chain reaction (PCR). Some authors recommend CSF (cerebrospinal fluid) analysis to perform PCR before and after treatment, since the CSF may be infected, despite not presenting clinical manifestations. If it is infected, treatment should be maintained until the CSF PCR becomes negative. A limitation of the PCR is if its positivity once the treatment is established is due to the persistence of the infection or to the persistence of DNA from the already dead bacilli.

The cultivation of the microorganism would be the best means to verify the effectiveness of eradication. It is expected that with the identification of a culture medium such as that recently described by some researchers we can know more about the microorganism, which is the most suitable antibiogram, and the most effective therapeutic option in those patients who do not respond to conventional Whipple's disease treatment. In this sense, a successful response has been described in a patient with a cerebral disease that did not respond to conventional treatment, to the combination of interferon gamma and cotrimoxazole.

Conclusion

Whipple’s disease without a proper treatment can be fatal. In this sense, it has been discovered that even with treatment, with duration of fewer than six months, there are risks of future recurrences. It is necessary to accomplish it for a year or more to get results and remission of the symptoms.

Also Read:

Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: September 4, 2018

This article does not provide medical advice. See disclaimer

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