Waterhouse-Friderichsen Syndrome

What is Waterhouse-Friderichsen syndrome?

Waterhouse-Friderichsen syndrome (WFS) is a life-threatening condition in which one or both of your adrenal glands stop working because of bleeding into the glands (adrenal hemorrhage), usually from an infection. Without treatment, this causes your adrenal glands to stop producing the hormone cortisol (adrenal crisis).

Another name for WFS is hemorrhagic adrenalitis.

How common is Waterhouse-Friderichsen syndrome?

Waterhouse-Friderichsen syndrome is rare. It accounts for about 1% of deaths in routine autopsies (medical examinations of a corpse to determine the cause of death).

What are the symptoms of Waterhouse-Friderichsen syndrome?

If a virus or bacteria cause WFS, you may suddenly develop infection symptoms. These include:

• Fever.
• Chills.
• Headache.
• Nausea and vomiting.
• Muscle pain, usually in your lower back, abdomen or legs.
• Joint pain.
• Loss of consciousness (syncope).

As bleeding affects your adrenal glands and other parts of your body, other symptoms may include:

• Disseminated intravascular coagulation (DIC).
• Skin rash.
• Septic shock.

What clinical symptoms are common with adrenal hemorrhage?

WFS associates with adrenal hemorrhage. Common adrenal hemorrhage symptoms include:

• Abdominal pain.
• Pain between hips and lower ribs (loins).
• Malaise (discomfort/uneasiness).
• Sluggishness.
• Nausea and vomiting.
• Diarrhea.

What causes adrenal hemorrhage?

Bacterial and viral infections (sepsis) are the most common causes of WFS. Bacterial causes may include:

• Pseudomonas aeruginosa.
• Meningococcus bacteria.
• Streptococcus pneumoniae.
• Haemophilus influenzae.
• Escherichia coli.
• Staphylococcus aureus.
• Group A beta-hemolytic Streptococcus.
• Capnocytophaga canimorsus.
• Enterobacter cloacae.
• Pasteurella multocida.
• Plesiomonas shigelloides.
• Neisseria gonorrhoeae.
• Moraxella duplex.
• Rickettsia rickettsii.
• Bacillus anthracis.
• Treponema pallidum.
• Legionella pneumophila.

Viral causes may include:

• Cytomegalovirus (CMV).
• Parvovirus.
• Epstein-Barr virus.
• Ebola virus disease.
• Varicella-zoster virus.

Other WFS risk factors may include:

• Anticoagulant medications.
• Thrombocytopenia.
• Damage to your adrenal glands.

Who does Waterhouse-Friderichsen syndrome affect?

Waterhouse-Friderichsen syndrome is more common in children than in adults.

What are the complications of Waterhouse-Friderichsen syndrome?

Without immediate treatment, Waterhouse-Friderichsen syndrome is fatal.

How is Waterhouse-Friderichsen syndrome diagnosed?

A healthcare provider can diagnose Waterhouse-Friderichsen syndrome. They’ll ask about your symptoms, review your medical history and perform a physical examination.

WFS can be challenging to diagnose because it resembles septic shock. To help confirm a WFS diagnosis, your provider will order tests.

What tests will be done to diagnose Waterhouse-Friderichsen syndrome?

Your healthcare provider may order the following tests to confirm an infection:

Imaging tests

An ultrasound or CT (computed tomography) scan can show pools of blood in your adrenal glands.

Blood tests

Blood tests can confirm a bacterial infection. During a blood test, a provider will use a thin needle (about the size of a standard earring post) to withdraw a small amount of blood, usually from a vein in your arm. They’ll look at your blood under a microscope to see if you have any signs of an infection.

Meningococcus bacteria tests

If your provider thinks that meningococcus bacteria are responsible for your WFS, they may recommend:

• Spinal tap (lumbar puncture). Your provider will insert a thin needle (about the size of a standard earring post or smaller) between two bones (vertebrae) in your lower back to withdraw and test a small amount of spinal fluid.
• Skin biopsy. If you have a rash, your provider will remove and test a small layer of skin.
• Gram stain. Your provider may collect other samples from a site of suspected infection to test for bacteria.
• Urinalysis. You’ll urinate (pee) into a special container. Your provider will then examine the sample for signs of infection.

Acute adrenal crisis tests

The following tests can help your provider properly diagnose an adrenal crisis:

• Adrenocorticotropic hormone (ACTH) stimulation test. Your provider will give you a shot of ACTH and take blood samples 30 to 60 minutes after the shot to see how well your adrenal glands respond to the ACTH.
• Blood sugar tests. Your provider will use a thin needle to withdraw a small amount of blood. They’ll then measure the amount of sugar (glucose) in your blood sample.
• Cortisol test. Your provider will take a blood, pee or saliva (spit) sample — or a combination of two to three — to determine how much cortisol your adrenal glands release.
• pH blood test. Your provider may use a thin needle to withdraw blood and conduct an arterial blood gas (ABG) test or electrolyte panel to measure your blood pH. The pH scale is the levels of acids and bases in your blood. It ranges from 0 (very acidic) to 14 (very basic or alkaline). A normal pH range in your blood is between 7.35 and 7.45.
• Potassium blood test. Your provider will use a thin needle to withdraw blood and measure the amount of potassium. Potassium is a nutrient in foods that helps your nerves and muscles work properly.
• Sodium blood test. Your provider will use a thin needle to withdraw blood and measure the amount of sodium. Sodium is a nutrient in many foods that helps your nerves and muscles work properly.

How is Waterhouse-Friderichsen syndrome treated?

If you have WFS, you’ll present with sepsis. Your healthcare provider will give you antibiotics right away as they order tests to determine the exact cause of your WFS. They’ll also monitor your electrolyte and water levels.

Once your provider manages your sepsis symptoms, they’ll treat you with glucocorticoid and mineralocorticoid medications. Glucocorticoids and mineralocorticoids are steroid hormones. Glucocorticoids help reduce inflammation. Mineralocorticoids help regulate your salt and water levels.

You may also need a blood transfusion or an angioembolization. An angioembolization combines an angiogram and embolization (closing off your blood vessels) to see and block off the arteries that supply blood to your adrenal glands.

Most people must take supplementary hormones for the rest of their lives after WFS.

How soon after treatment will I feel better?

Most people will start to feel better about a week and a half after treatment. However, you may continue to have physical or emotional symptoms for months or even years after you recover. These may include:

• Lack of appetite.
• Fatigue.
• Difficulty sleeping (insomnia).
• Getting sick more often.
• Anxiety.
• Depression.

Can Waterhouse-Friderichsen syndrome be prevented?

You can lower your risk of developing Waterhouse-Friderichsen syndrome by:

• Thoroughly washing your hands to prevent infections.
• Getting a meningococcal bacteria vaccine.
• Treating a suspected infection immediately.

What can I expect if I have Waterhouse-Friderichsen syndrome?

Without treatment, WFS is fatal.

With prompt, proper treatment and rehabilitation, many people who have WFS recover. However, you may require intensive medical care, and WFS effects may last months or even years.

How do I take care of myself?

You and your healthcare provider will work together to develop the safest and most effective rehabilitation plan for you as you recover. Your plan may include:

• Resting.
• Following a balanced diet that’ll help you regain your strength. It’s a good idea to talk to a nutritionist.
• Setting small goals, such as sitting up, standing, dressing yourself, taking a shower, walking short distances or climbing stairs.
• Talking about your feelings to your family, friends or therapist.
• Exercising as you regain strength and energy.

When should I see my healthcare provider?

Talk to your healthcare provider if you’re not feeling better after treatment or can’t meet rehabilitation goals.

When should I go to the ER?

WFS is a severe condition that’s fatal without treatment. If you have any symptoms of WFS, including an infection that’s not improving or appears to be getting worse, go to the emergency room immediately.

What questions should I ask my healthcare provider?

• How do you know that I have Waterhouse-Friderichsen syndrome?
• If I don’t have Waterhouse-Friderichsen syndrome, what other condition might I have?
• What’s the cause of my Waterhouse-Friderichsen syndrome?
• Will I need surgery to repair my adrenal glands?
• Do you think I’ll make a full recovery?
• Do I need to take supplementary hormones?
• How long will I need to take supplementary hormones?

A note from Cleveland Clinic

Waterhouse-Friderichsen syndrome is a severe condition that needs immediate medical care. If you have any symptoms of WFS or an infection that won’t go away, it’s important to get to an emergency room as soon as possible. Follow all of your healthcare provider’s treatment recommendations to manage WFS and recover as quickly as possible.