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Waterhouse-Friderichsen syndrome

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 number inside the body. Symptoms include:

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

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

Bleeding into the adrenal glands causes not enough adrenal hormones to be produced. 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

The 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:

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

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

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.

Gallery

Meningococcal lesions on the back
Meningococcemia is a life-threatening infection that occurs when the bacteria Neisseria meningitidis invades the blood stream. Bleeding into the skin (petechiae and purpura) usually occurs and the tissue may die (become necrotic or gangrenous). If the patient survives, the areas heal with scarring.
Meningococcal lesions on the back
Meningococcemia is a life-threatening infection that occurs when the bacteria Neisseria meningitidis invades the blood stream. Bleeding into the skin (petechiae and purpura) usually occurs and the tissue may die (become necrotic or gangrenous). If the patient survives, the areas heal with scarring.

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; 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.

Last reviewed September 1, 2021 by Jatin M. Vyas, MD, PhD, Associate Professor in Medicine, Harvard Medical School; Associate in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team..

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