Definition

Waterhouse-Friderichsen syndrome (WFS) is a group of symptoms caused when the adrenal glands fail to function normally. This occurs as a result of bleeding into the glands.

Alternative Names

Fulminant meningococcemia - Waterhouse-Friderichsen syndrome; Fulminant meningococcal sepsis - Waterhouse-Friderichsen syndrome; Hemorrhagic adrenalitis

Causes

The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney. The adrenal glands produce and release different hormones that the body needs to function normally. The adrenal glands can be affected by many diseases, such as infections like WFS.

WFS is caused by severe infection with meningococcus bacteria or other bacteria such as:

• Group B streptococcus
• Pseudomonas aeruginosa
• Streptococcus pneumoniae
• Staphylococcus aureus

Symptoms

Symptoms occur suddenly. They are due to the bacteria growing in large numbers inside the body. Symptoms include:

• Fever and chills
• Joint and muscle pain
• Headache
• Vomiting

The infection with bacteria causes bleeding throughout the body, which causes:

• A rash throughout the body
• Disseminated intravascular coagulation in which small blood clots cut off blood supply to the organs
• Septic shock

Bleeding into the adrenal glands makes the glands unable to produce enough adrenal hormones. This is called adrenal crisis, and it leads to symptoms such as:

• Dizziness, weakness
• Very low blood pressure
• Very fast heart rate
• Confusion or coma

Exams and Tests

Your health care provider will perform a physical exam and ask about your symptoms.

Blood tests will be done to confirm a bacterial infection. Tests may include:

• Blood culture
• Complete blood count with differential
• Blood clotting studies

If your provider suspects the infection is caused by meningococcus bacteria, other tests that may be done include:

• Lumbar puncture to get a sample of spinal fluid for culture
• Skin biopsy and Gram stain
• Urine analysis

Tests that may be ordered to help diagnose acute adrenal crisis include:

• ACTH (cosyntropin) stimulation test
• Cortisol blood test
• Blood sugar
• Potassium blood test
• Sodium blood test
• Blood pH test

Treatment

Antibiotics are started right away to treat the bacterial infection. Glucocorticoid medicines will also be given to treat adrenal gland insufficiency. Supportive treatments will be needed for other symptoms.

Outlook (Prognosis)

WFS is fatal unless treatment for the bacterial infection is started right away and glucocorticoid drugs are given.

Prevention

To prevent WFS caused by meningococcal bacteria, a vaccine is available.

References

Newell-Price JDC, Auchus RJ. The adrenal cortex. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 15.

Stephens DS. Neisseria meningitides. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 211.