Waterhouse-Friderichsen syndrome

Definition

Waterhouse-Friderichsen syndrome (WFS) is a group of symptoms resulting from the failure of the adrenal glands to function normally as a result of bleeding into the gland.

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 (multiplying) inside the body. Symptoms include:

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

Infection with bacteria causes bleeding throughout the body, which causes:

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

Bleeding into the adrenal glands causes adrenal crisis, in which enough adrenal hormones are not produced. This leads to symptoms such as:

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

Exams and Tests

The health care provider will perform a physical examination and ask about the person's 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 the 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.

Possible Complications

Prevention

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

Images

References

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 Saunders; 2020:chap 211.

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.