Medical Information
Whipple’s Disease Lymphadenopathy

Whipple’s Disease Lymphadenopathy

Brain & Nerve
Bone, Muscle, & Joint
Contributed byMaulik P. Purohit MD MPHDec 25, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Enlarged Lymph Nodes due to Whipple’s Disease
  • Whipple’s Disease causing Enlarged Lymph Nodes
  • Whipple’s Disease Lymphadenitis

What is Whipple’s Disease Lymphadenopathy? (Definition/Background Information)

  • Whipple’s Disease Lymphadenopathy is the inflammation and enlargement of lymph nodes due to underlying Whipple’s Disease, a rare infection that is caused by the bacterium Tropheryma whipplei
  • The infection can affect different parts of the body, such as the joints, brain, lung, eyes, heart, and other regions. The signs and symptoms of Whipple’s Disease Lymphadenopathy include enlarged abdominal and axillary lymph nodes that are not painful
  • The underlying Whipple’s Disease can cause a variety of signs and symptoms including poor absorption of food from the gastrointestinal tract, weight loss, chronic non-bloody diarrhea, and frequent joint pain
  • This bacterial infection is treated using antibiotics. The prognosis of Whipple’s Disease Lymphadenopathy generally depends upon the severity of the underlying signs and symptoms due to the bacterial infection

Who gets Whipple’s Disease Lymphadenopathy? (Age and Sex Distribution)

  • Whipple’s Disease Lymphadenopathy occurs due to underlying Whipple’s Disease, which is usually diagnosed in middle-aged and older individuals (median age of diagnosis is 49 years)
  • The infection affects both genders, though males are affected much more than females (male-female ratio is 8:1)
  • Caucasians are affected the most, when compared to other races and ethnic groups
  • Even though it is an uncommon condition, Whipple’s Disease occurs around the world

What are the Risk Factors for Whipple’s Disease Lymphadenopathy? (Predisposing Factors)

The main risk factor for Whipple’s Disease Lymphadenopathy is underlying Whipple’s Disease. The risk factors for Whipple’s Disease include:

  • Individuals with a decreased immune function are at an increased risk
  • Caucasians
  • Genetic factors present in family history
  • Farmers and agriculturist The disease commonly occurs in farmers and individuals, who are frequently exposed to soil and animals
  • Sewage plant workers

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Whipple’s Disease Lymphadenopathy? (Etiology)

Tropheryma whipplei is a type of bacterium that causes the infectious Whipple’s Disease and consequently Whipple’s Disease Lymphadenopathy.

  • The T. whipplei bacteria attack the intestinal lining and interferes with the ability of the intestines to absorb nutrients properly
  • Genetic factors that may affect an individual’s immune system can be another reason for Whipple’s Disease

What are the Signs and Symptoms of Whipple’s Disease Lymphadenopathy?

In a minority of individuals, the infection may be asymptomatic. In others, the signs and symptoms of Whipple’s Disease Lymphadenopathy may include:

  • Lymphadenopathy or enlarged lymph nodes
  • It usually involves the lymph nodes of the abdomen, armpits (axillary), and head and neck region (cervical region)
  • However, other lymph nodes of the body may also be affected
  • The swollen lymph nodes are generally painless

The associated signs and symptoms of Whipple’s Disease depend upon the body systems affected. It may get better or worse with time and may include:

Gastrointestinal (GI) symptoms:

  • Pain in the abdomen
  • Diarrhea
  • Weight loss (seen in almost 90% of the cases), due to loss of appetite
  • Distension - increase in pressure and volume in the abdomen
  • Steatorrhea - presence of excess fat in feces
  • GI bleeding

Musculoskeletal and skin symptoms:

  • Polyarthralgia - pain and aches in many joints
  • Hyperpigmentation - darkening of the skin, due to increase in melamine

Cardiac symptoms:

  • Pericarditis - inflammation of the sac-like covering, around the heart
  • Myocarditis - inflammation of the heart muscles
  • Injury in the valves

Between 5-40% of the individuals have central nervous system signs and symptoms that include:

  • Headache, confusion, dementia
  • Ophthalmoplegia - vision problems in lateral gaze
  • Myoclonus - sudden jerking of a muscle or a group of muscles
  • Oculomasticatory myorhythmia - unique movement disorder affecting the eyes and mouth muscles
  • Gait abnormalities - deviation from normal walking
  • Seizures, brain disorders
  • Coma, state of unconsciousness

Ocular symptoms:

  • Uveitis - swelling of the uvea, the middle layer of the eye
  • Vitritis - inflammation of the jelly, present in the rear side of the eye
  • Keratitis - inflammation of the cornea, in the front portion of the eye
  • Retinitis - inflammation of the retina, possibly leading to blindness
  • Retinal hemorrhages - eye disorder, where bleeding occurs in the back of the eye

Pulmonary symptoms:

  • Pleural effusion - accumulation of excess fluid around the lungs
  • Stretching of the mediastinum

Other non-specific signs and symptoms may include:

  • Chest pain
  • Mild fever
  • Chronic cough
  • Anemia and blood clotting related abnormalities

How is Whipple’s Disease Lymphadenopathy Diagnosed?

A case of Whipple’s Disease Lymphadenopathy may be diagnosed using the following methods:

  • A complete physical examination and a thorough medical history
  • The physician may order complete blood count, blood albumin level, and stool sample tests to help in the diagnosis of Whipple’s Disease

The gold standard for the diagnosis of Whipple’s Disease is a tissue biopsy of the duodenum, during an upper gastrointestinal endoscopy. Examination of the duodenal biopsy will show PAS-positive, non-acid fast organisms, within the macrophages.

  • Molecular tests, such as polymerized chain reaction (PCR) can be performed on a variety of specimens, such as saliva, stool, gastric fluid, and intestinal fluid
  • It is important to note that a positive PCR test, in the absence of signs and symptoms does not indicate Whipple’s Disease. This could be due to the fact that the bacteria causing Whipple’s Disease may be found in normal healthy individuals, without causing any infection
  • A negative PCR test would rule out the possibility of the disease

Note: Molecular studies have shown that individuals without signs and symptoms of Whipple’s Disease have the DNA of the causative bacterium Tropheryma whipplei. This indicates that the bacteria may be more prevalent in humans than previously believed. Further, the presence of the bacteria does not always result in signs and symptoms of the infection.

  • Test for immunoglobulins: Presence of antibodies specific to bacteria may be present in blood (IgM and IgG antibodies)
  • Rarely, electron microscopy can be performed
  • Biopsy of the lymph nodes can also be performed, which may demonstrate the presence of the bacteria within the lymph nodes

In case of a lymph node biopsy, the following information may be noted:

  • Lymph node biopsy: A lymph node biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. The pathologist may use special techniques, such as immunohistochemistry stains, to help reveal the presence of the pathogen
  • The lymph node biopsy may be performed through any of the following procedures:
    • Fine needle aspiration biopsy (FNAB): A device called a cannula is used to extract tissue or fluid from the lymph nodes
    • Lymph node core biopsy of the enlarged lymph node
    • Lymph node open biopsy of the enlarged lymph node

A differential diagnosis may be performed to exclude other conditions before arriving at a diagnosis such as:

  • Lymph node enlargement due to Mycobacterium tuberculosis
  • Lymph node enlargement due to Mycobacterium avium-intracellulare
  • Lymphoma of abdominal lymph nodes

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Whipple’s Disease Lymphadenopathy?

The complications from Whipple’s Disease causing Lymphadenopathy could include the following: 

  • Longstanding infection that may be recurrent
  • The infection may block the lymphatics in the abdomen causing the lymphatic vessel to rupture. This can result in the accumulation of fluid in the abdominal cavity, called chylous ascites
  • Enlarged liver (hepatomegaly)
  • Involvement of the bone marrow, which is seen in up to 40% of the affected individuals
  • Damage to the heart walls causing murmurs and heart dysfunction
  • Nutritional deficiencies, due to poor absorption of vitamins
  • Severe weight loss and malnutrition
  • Brain damage

How is Whipple’s Disease Lymphadenopathy Treated?

A treatment of Whipple’s Disease Lymphadenopathy is undertaken using antibiotic therapy. Generally, antibiotic therapy should be administered for a prolonged period of time (1-2 years). The following antibiotics may be given:

  • Intravenous ceftriaxone
  • Administering sulfamethoxazole-trimethoprim or SMX-TMP, for a period of 1-2 years
  • When the infection is severe:
    • If the patient exhibits neurologic symptoms, oral doxycycline in combination with the anti-malarial drug hydroxychloroquine is administered
    • The antibiotics are prescribed for a long-term
    • Vitamin and mineral supplements may be given to compensate for the poor absorption of nutrients
  • Studies have shown that treatment courses less than 1-year would result in the condition recurring in about 40% of the individuals
  • Undertaking treatment of underlying immunosuppression

With appropriate treatment, the swollen lymph nodes and other signs and symptoms of the condition should get better.

How can Whipple’s Disease Lymphadenopathy be Prevented?

Current medical research has not established a way of preventing Whipple’s Disease Lymphadenopathy caused by underlying bacterial infection due to T. whipplei.

  • Medical screening at regular intervals with scans and physical examinations, are advised for those who have already endured Whipple’s Disease
  • Farmers and sewage plant workers should contact their primary care physician early, if they are experiencing any of the symptoms of the disease

What is the Prognosis of Whipple’s Disease Lymphadenopathy? (Outcomes/Resolutions)

The prognosis of Whipple’s Disease Lymphadenopathy is based on the severity of the underlying bacterial infection

  • In general, the prognosis of Whipple’s Disease is excellent with appropriate antibiotic treatment
  • Without treatment, the condition can be fatal

Additional and Relevant Useful Information for Whipple’s Disease Lymphadenopathy:

General information on lymph nodes:

The lymph nodes are part of one’s immune system. The lymph nodes are present throughout the body, usually in groups. In normal healthy adults, one cannot feel (see or touch) the lymph nodes readily. However, if they are enlarged, they can be felt either by the individual himself/herself or by the healthcare provider.

Enlarged lymph nodes can occur in both benign and malignant conditions. Hence, the cause of enlarged lymph nodes should be evaluated. If no obvious cause for enlargement of lymph nodes is found, then the possibility of a lymph node malignancy should be ruled out.

In a majority of individuals, a lymph node swelling is caused by a benign process such as an inflammation or infection. In many cases, swollen lymph nodes are part of other signs and symptoms. This can help a healthcare provider arrive at a list of follow-up tests and ensure an accurate diagnosis. Superficial enlarged lymph nodes can be felt by the healthcare provider through palpation. Enlarged lymph nodes deep in the body are often detected by radiological studies such as X-rays, ultrasound scan, CT and MRI scans.

Some enlarged lymph nodes can be painful, while others may be painless. Depending upon the underlying cause, enlarged lymph nodes can be localized to a particular area of the body, or they can be generalized, meaning that they are present throughout the body.

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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team


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