Whipples disease involves a rare form of bacterial infection that primarily affects a persons small intestine.
Signs of Whipple's Disease
The signs and symptoms of Whipple's disease can vary rather widely. The more classic signs and symptoms include periodic joint pain, either with or without inflammation, which may persist for years prior to the appearance of any other symptoms. A person can experience chronic diarrhea, with or without the presence of blood. They may experience a loss of weight, as well as abdominal pain and bloating. Fever and fatigue, as well as anemia are also classic signs of Whipple's disease.
Some of the less common signs and symptoms of Whipple's disease include a darkening of the person's skin, and enlargement of the lymph nodes. The person may experience a chronic cough, as well as chest pain and inflammation of the membrane surrounding their heart, known as, 'pericarditis.' The person may even experience heart failure.
There are also some neurologic symptoms that can occur in some persons with Whipple's disease. These symptoms include things such as vision problems, dementia, or headaches. The person might also experience symptoms such as facial numbness, muscle weakness or twitching, difficulty with walking, or memory issues. All of these symptoms can mimic other forms of neurologic conditions. Symptoms of neurologic, heart, or lung disease appear on occasion, without gastrointestinal symptoms.
Causes of Whipple's Disease
The bacteria Tropheryma whipplei, also referred to as, 'T. whipplei,' causes Whipple's disease. T. whipplei may cause internal sores called lesions, as well as the thickening of tissues. 'Villi,' or tiny, finger-like projections which line a person's small intestine, present an abnormal and club-like appearance. Damaged intestinal lining fails to appropriately absorb nutrients, resulting in both malnutrition and diarrhea.
Medical science is not sure how the T. whipplei bacteria infects people, although one theory suggest that some people are more vulnerable to the disease; most likely due to genetic factors which influence their body's immune system. The theory is supported by the existence of a number of asymptomatic carriers, or people who have the T. whipplei bacteria present in their bodies, yet do not become ill. There is also the fact that the bacteria are more common in the environment. They are present in both sewage and soil, something that does not agree with the rareness of the disease in people. While there have been multiple cases of Whipple's disease that have occurred in the same family, there is no documentation in existence of a person-to-person transmission.
Diagnosing Whipple's Disease
Due to the fact that Whipple's disease is rare, a doctor might attempt to first rule out more common forms of conditions that present similar symptoms. Some of the conditions a doctor may try to rule out include things such as inflammatory rheumatic disease, celiac disease, intra-abdominal lymphoma, neurologic conditions, or Mycobacterium in person with AIDS. A diagnosis of Whipple's disease is made through a careful evaluation of the person's symptoms, an endoscopy, as well as a biopsy with tissue straining. Electron microscopy and polymerase chain reaction (PCR) testing are used in order to confirm the diagnosis.
Endoscopy is used to examine the lining of a person's small intestine. An, 'endoscope,' is a a thin, flexible, lighted tube with a small camera on the tip. An endoscope is inserted through the person's mouth and stomach and into their small intestine. The endoscope sends images that are taken inside the person's small intestine to a video monitor, where a health care provider can view them.
During the endoscopy, a biopsy is performed to collect samples of the tissue lining the person's small intestine for examination using staining. The staining process may reveal T. whipplei-infected cells from tissues when they are viewed with a light microscope. Due to the fact that the staining process is nonspecific, meaning that it may also stain cells that are infected with other forms of bacteria or fungi, a number of doctors opt to confirm the results with a second test - such as either PCR testing, or electron microscopy.
Electron microscopy has a greater resolution than light microscopy and can be used to see T. whipplei bacteria inside infected cells that have been taken through a biopsy. The T. whipplei bacteria have a rather unique appearance which can be easily identified. PCR testing may both detect and identify very low levels of bacterial DNA in body fluids and tissues. If T. whipplei DNA is present in the person's cerebrospinal fluid, it is an indication of neurologic Whipple's disease. PCR testing use for Wipple's disease is still fairly new, so the results are often supported by staining or electron microscopy.
Treatment of Whipple's Disease
Long-term administration of antibiotics are used to treat Whipple's disease. The standard therapy for Whipple's disease involves an initial treatment with intravenous antibiotics over a two-week period, followed by daily oral administration of antibiotics for one to two years. The antibiotics used to treat Whipple's disease can include ceftriaxone and penicillin G plus streptomycin. A combination oral antibiotic known as Trimethoprim/sulfamethoxazole which has the ability to enter a person's cerebrospinal fluid and brain is often used to treat Whipple's disease as well. There is an alternative treatment for Whipple's disease through a combination of Doxycycline plus Hydroxychloroquine taken over a period of twelve to eighteen months. The approach is recommended for people who have neurologic Whipple's disease who also take long-term antibiotics which may enter their cerebrospinal fluid and brain.
Once a person has received treatment for Whipple's disease, their likely outcome is good. The majority of the symptoms of the disease disappear within about a month. The potential for a relapse is common, and this potential highlights the need for close observation for the return of any symptoms. An endoscopy with small intestinal biopsy, followed by staining and either PCR testing or electron microscopy should be repeated one year after the person receives treatment for Whipple's disease.
People who experienced neurologic Whipple's disease and have a relapse tend to have poorer health outcomes. They may experience serious neurologic symptoms, or even death. Some scientists argue that every case of Whipple's disease should be considered to be neurologics. People who experience a relapse of neurologic Whipple's disease are sometimes treated with a combination of both antibiotics and weekly injections of interferon gamma. Interferon gamma is a substance that is made by the body which activates their immune system.
There are some important things to remember about Whipple's Disease:
Scientists are unsure how T. whipplei infects people
Whipple's disease is treated with long-term antibiotics
Whipple's disease is most common in middle-aged Caucasian men
Whipple's disease can also affect the heart, lungs, brain, joints, and eyes
Bacteria called Tropheryma whipplei (T. whipplei) cause Whipple's disease
After treatment, the likely outcome for most people with Whipple's disease is good
Whipple's disease is a rare bacterial infection primarily affecting the small intestine
People with neurologic Whipple's disease who relapse tend to have much poorer health outcomes
Electron microscopy and polymerase chain reaction (PCR) testing are used to confirm a diagnosis
Neurologic symptoms of Whipple's disease can mimic those of almost any other neurologic condition
Whipple's disease is diagnosed through a careful evaluation of symptoms, endoscopy, and biopsy with tissue staining
Classic signs and symptoms of Whipple's disease include joint pain, chronic diarrhea, weight loss, abdominal pain and bloating, fever, fatigue, and anemia