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Whipple disease

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Other Names:
Intestinal lipodystrophy; Intestinal lipophagic granulomatosis; Secondary Non-tropical Sprue; Intestinal lipodystrophy; Intestinal lipophagic granulomatosis; Secondary Non-tropical Sprue; Tropheryma whippelii infection See More
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Whipple disease is an infectious bacterial disease that affects many different organ systems and interferes with the body's ability to process (metabolize) fats.[1] The disease usually occurs in the gastrointestinal system, but may affect any part of the body including the heart, lungs, brain, joints, and eyes.[2] In the gastrointestinal system, it interferes with the body's ability to absorb certain nutrients.[2][3] This leads to a condition known as malabsorption.[3] Whipple disease causes weight loss, incomplete breakdown of carbohydrates or fats, and problems with the immune system.[2] It is caused by infection from bacteria called Tropheryma whipplei.[2][3] When recognized and treated, Whipple disease can usually be cured. Untreated, the disease may be fatal.[2]
Last updated: 3/23/2017
Symptoms of Whipple disease can vary from person to person and may affect many systems of the body. Some of the most common symptoms, occurring in the majority of people with Whipple disease, include:[4]
  • Weight loss.
  • Diarrhea.
  • Joint pain.

Other signs and symptoms may include:[1][2][4]

  • Neurological symptoms such as eye and facial muscle abnormalities, confusion, seizures, ataxia, memory loss, and vision impairment.
  • Fatigue.
  • Weakness.
  • Abdominal bleeding.
  • Abdominal pain.
  • Fever.
  • Loss of appetite.
  • Darkening of the skin
 
Last updated: 4/23/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 47 |
Medical Terms Other Names
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HPO ID
80%-99% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Anorexia 0002039
Arthralgia
Joint pain
0002829
Arthritis
Joint inflammation
0001369
Cachexia
Wasting syndrome
0004326
Depressivity
Depression
0000716
Developmental regression
Loss of developmental milestones
Mental deterioration in childhood
[ more ] 		0002376
Diarrhea
Watery stool
0002014
Fatigue
Tired
Tiredness
[ more ] 		0012378
Fever 0001945
Malabsorption
Intestinal malabsorption
0002024
Mediastinal lymphadenopathy
Swollen lymph nodes in center of chest
0100721
Myoclonus 0001336
30%-79% of people have these symptoms
Hepatomegaly
Enlarged liver
0002240
Hypotension
Low blood pressure
0002615
Myalgia
Muscle ache
Muscle pain
[ more ] 		0003326
Pleuritis
Inflammation of tissues lining lungs and chest
0002102
Sleep disturbance
Difficulty sleeping
Trouble sleeping
[ more ] 		0002360
Splenomegaly
Increased spleen size
0001744
Uveitis 0000554
5%-29% of people have these symptoms
Abnormal pyramidal sign 0007256
Anemia
Low number of red blood cells or hemoglobin
0001903
Ataxia 0001251
Chest pain 0100749
Cough
Coughing
0012735
Cranial nerve paralysis 0006824
Encephalitis
Brain inflammation
0002383
Erectile dysfunction
Abnormal erection
Erectile abnormalities
[ more ] 		0100639
Galactorrhea
Spontaneous milk flow from breast
0100829
Gastrointestinal hemorrhage
Gastrointestinal bleeding
0002239
Generalized hyperpigmentation 0007440
Hydrocephalus
Too much cerebrospinal fluid in the brain
0000238
Hyponatremia
Low blood sodium levels
0002902
Hypothyroidism
Underactive thyroid
0000821
Increased intracranial pressure
Rise in pressure inside skull
0002516
Insulin resistance
Body fails to respond to insulin
0000855
Muscle weakness
Muscular weakness
0001324
Myocardial infarction
Heart attack
0001658
Myocarditis
Inflammation of heart muscle
0012819
Myositis
Muscle inflammation
0100614
Pedal edema
Fluid accumulation in lower limbs
Lower leg swelling
[ more ] 		0010741
Pericarditis
Swelling or irritation of membrane around heart
0001701
Peripheral neuropathy 0009830
Polydipsia
Extreme thirst
0001959
Proptosis
Bulging eye
Eyeballs bulging out
Prominent eyes
Prominent globes
Protruding eyes
[ more ] 		0000520
Respiratory insufficiency
Respiratory impairment
0002093
Seizure 0001250
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Last updated: 7/1/2020
Whipple disease is caused by infection from bacteria called Tropheryma whipplei (or Tropheryma whippelii).[2][3] It is not known how people become infected with the bacteria, but there is no evidence of human to human transmission.[5]

It seems that these bacteria are common in soil or water, and many reports of disease occur in patients who work in trades where they have frequent contact with soil such as agriculture and construction. The bacteria may also be part of the normal flora of the body.[5] One study revealed its presence in saliva in 35% of a sample of 40 healthy patients.[4] These findings, and the fact that Whipple disease is more common in people who have an antigen known as HLA-B27 suggest that there may be a genetic predisposition (susceptibility) in those people with the disease, resulting in an abnormal host response to a microorganism that occurs frequently in humans.[4][5]  In addition, most, if not all patients, have an immune defect which make it harder to fight infections.[5] 

 

Last updated: 3/23/2017
The standard treatment for Whipple disease is a prolonged course of antibiotics to destroy the bacteria that cause the disease.[1][2][3] A number of different types, doses, and schedules of antibiotics may be used in an effort to find the best treatment.[2]

A medicine called ceftriaxone 2g, or penicillin G are commonly given intravenously as a first option. These antibiotics are typically followed by another antibiotic (such as trimethoprim-sulfamethoxazole) taken by mouth for up to 1 year. If symptoms come back during antibiotic use, the antibiotic treatment may be changed.[3]
Depending on the seriousness of the disease, treatment may also include fluid and electrolyte replacement. Electrolytes are salts and other substances in body fluid that the heart and brain need to function properly.[2] Extra iron, folate, vitamin D, calcium, and magnesium may also be given to help compensate for the vitamins and minerals the body cannot absorb on its own.[2][3]

People who are being treated for Whipple disease should be closely monitored by a health care provider because signs of the disease can return after therapy has been finished.[3] Full recovery of the small intestine may take up to 2 years, but the symptoms usually disappear in less time. Because relapse is common even after successful treatment, the health care team may continue to monitor the patient for many years.[2]

In general, surgery is not needed, however, neurosurgical care is important for both obtaining diagnostic biopsy specimens in selected patients and placement of ventriculoperitoneal shunting in patients with hydrocephalus.[6] 
Last updated: 3/23/2017

After treatment, the likely outcome for most people with Whipple disease is good. Most symptoms disappear in about 1 month. Relapse is common, however, highlighting the need to closely watch for a return of symptoms. Endoscopy with small intestinal biopsy followed by PAS staining and electron microscopy or PCR testing should be repeated 1 year after the start of treatment.[2]

People with neurologic Whipple disease who relapse tend to have much poorer health outcomes, including serious neurologic symptoms and even death; therefore, some researchers argue that all cases of Whipple disease should be considered neurologic.[2]

Prompt diagnosis is imperative, as very effective therapies are easy to employ with typically rapid limitation of central nervous system (CNS) progression and even partial reversal of CNS symptoms. If left untreated, progression to death may come as quickly as 1 month after CNS involvement begins.[3]

Last updated: 12/8/2015

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
The differential diagnosis includes inflammatory bowel disease, malabsorption syndrome, infectious diarrhea, mesenteric lymphadenitis, seronegative polyarthritis, soft tissue rheumatism, culture-negative endocarditis, vasculitis, lymphoma, cerebrovascular disease, demential processes, HIV infection, atypical mycobacteriosis, sarcoidosis, unclear cutaneous symptoms, exophthalmos and many others.
Visit the Orphanet disease page for more information.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Whipple disease. Click on the link to view a sample search on this topic.

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  1. Whipple's Disease Information Page. National Institute of Neurological Disorders and Stroke (NINDS). https://www.ninds.nih.gov/Disorders/All-Disorders/Whipples-Disease-Information-Page. Accessed 6/5/2017.
  2. Whipple Disease. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). August 2014; http://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/whipple-disease/Pages/facts.aspx.
  3. Whipple's disease. MedlinePlus. 2016; http://www.nlm.nih.gov/medlineplus/ency/article/000209.htm.
  4. Roberts IM. Whipple Disease. Medscape Reference. December 30, 2017; http://emedicine.medscape.com/article/183350-overview#a7.
  5. Tropheryma whipelii. Department of Microbiology. Mount Sinai Hospital. https://eportal.mountsinai.ca/Microbiology//bug/TW/tw-bug.shtml.
  6. Bobustuc GC. CNS Whipple Disease Treatment & Management. Medscape Reference. December 2014; http://emedicine.medscape.com/article/1166639-treatment.