What's Up Doc? Whipple disease is not related to the Whipple procedure for pancreatic cancer

Q: What is Whipple disease and is it treated with a Whipple procedure?

A: Whipple procedure (also known as a pancreaticoduodenectomy) is named for Allen Whipple, a surgeon who in the mid-1930’s pioneered an operation to remove the head of the pancreas, part of the duodenum (the first part of the small intestine), the gallbladder and the bile duct, a complex operation used to treat certain cancers (such as pancreatic cancer of the head of the pancreas) and other conditions.

Not only is Whipple procedure NOT related to Whipple disease (WD), but George Whipple (for whom the disease is named) was not related to Allen Whipple, although apparently they were friends.

George Whipple had a patient in 1907 who had gastrointestinal (GI) issues (stool changes, abdominal pain, weight loss, absorption issues causing nutritional deficiencies, other) and arthritis in multiple joints. Dr. Whipple eventually suspected it could be due to an infection (having noted rod-shaped organisms in the patient’s intestines), and in 1949 when the condition was further studied, it was named WD. It was not until 1991 that the specific bacteria causing the condition was identified.

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The cause of WD is the bacteria Tropheryma whipplei, which is ubiquitous in the environment and is commonly found in people’s stool and other fluids (one study found DNA evidence of the bacteria in the saliva of a third of healthy patients). It is unclear why in rare causes the bacteria causes WD; it is thought that affected patients may have an issue with their immune system which allows this common bacteria to start to cause problems.

So, what is WD? It is a rare (there have only been a thousand cases or so reported, for an incidence of less than 1 in a million, although it is possible the disease is underdiagnosed and hence underreported) chronic bacterial infection (along with a systemic inflammatory response that is a key factor in the condition) that affects the GI tract (causing stool changes, abdominal pain, weight loss, nutrient absorption issues, and/or other symptoms), joints (causing swelling, inflammation and/or pain, most commonly in the ankles, knees and/or wrists), systemic issues (like fatigue, weakness, fever, other) and possibly other body organs:

• the heart by causing endocarditis and/or other conditions

• the brain causing a dementia-like confusion, walking/balance issues, and/or other

• the lungs causing interstitial lung disease, fluid around the lungs (pleural effusion) and/or other

• the skin, for example affecting the patient’s skin pigmentation

• the eyes, for example affecting vision and/or eye movements

• other symptoms.

The symptoms of WD are chronic and develop over years, with the specific manifestations changing over time, for example spontaneously worsening/improving, affecting different organs, etc.

The diagnosis of WD is suspected based on the patient’s symptoms, especially if they have the four "classic" symptoms of joint symptoms (arthralgia and/or arthritis), weight loss, chronic diarrhea and abdominal pain. However, many of these symptoms are pretty common, especially in older patients, and most commonly come from other conditions (for example joint issues from different types of arthritis, GI symptoms from many different causes, etc.). Therefore, the first step in evaluating patients with these symptoms is to look for the more common possible causes.

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Once other causes have been ruled out, the diagnosis of WD can be pursued and potentially verified by analyzing fluid/tissue from the patient’s affected organs (for example a biopsy of the small intestines, fluid from a joint effusion, etc.) utilizing polymerase chain reaction (PCR) testing.

Untreated (for example if it not definitively diagnosed), WD can be fatal. Once the diagnosis is made, WD is treated with antibiotics (often an initial aggressive regimen of intravenous antibiotics followed by oral antibiotics for a long duration, e.g., a year). Clinical/symptomatic improvement with appropriate treatment occurs in almost three-quarters of patients and is often very dramatic, although some issues (for example certain neurological symptoms) may be irreversible. Even when there is a good response, a relapse of the condition is possible and occurs in up to a third of patients, requiring a new round of treatment.

Jeff Hersh, Ph.D., M.D., can be reached at DrHersh@juno.com.

This article originally appeared on MetroWest Daily News: Whipple disease affects the GI tract and can cause serious problems