Waterhouse–Friderichsen syndrome

Waterhouse–Friderichsen syndrome (WFS) or hemorrhagic adrenalitis or Fulminant meningococcemia, is defined as adrenal gland failure due to bleeding into the adrenal glands, caused by severe bacterial infection (most commonly the meningococcus Neisseria meningitidis).^[1]

The bacterial infection leads to massive hemorrhage into one or (usually) both adrenal glands.^[2] It is characterized by overwhelming bacterial infection meningococcemia leading to massive blood invasion, organ failure, coma, low blood pressure and shock, disseminated intravascular coagulation (DIC) with widespread purpura, rapidly developing adrenocortical insufficiency and death.

Signs and symptoms

WFS is the most severe form of meningococcal septicemia. The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Soon a rash appears; first macular, not much different from the rose spots of typhoid, and rapidly becoming petechial and purpuric with a dusky gray color. Low blood pressure (hypotension) is the rule and rapidly leads to septic shock. The cyanosis of extremities can be impressive and the patient is very prostrated or comatose. In this form of meningococcal disease, meningitis generally does not occur. There is hypoglycemia with hyponatremia and hyperkalemia, and the ACTH stimulation test demonstrates the acute adrenal failure. Leukocytosis need not to be extreme and in fact leukopenia may be seen and it is a very poor prognostic sign. C-reactive protein levels can be elevated or almost normal. Thrombocytopenia is sometimes extreme, with alteration in prothrombin time (PT) and partial thromboplastin time (PTT) suggestive of diffuse intravascular coagulation (DIC). Acidosis and acute renal failure can be seen as in any severe sepsis. Meningococci can be readily cultured from blood or CSF, and can sometimes be seen in smears of cutaneous lesions. Dysphagia, atrophy of the tongue, and cracks at the corners of the mouth are also characteristic features.

Causes

Multiple species of bacteria can be associated with the condition:

• Meningococcus is another term for the bacterial species Neisseria meningitidis; blood infection with said species usually underlies WFS. While many infectious agents can infect the adrenals, an acute, selective infection is usually Meningococcus.
• Pseudomonas aeruginosa can also cause WFS.^[3]
• WFS can also be caused by Streptococcus pneumoniae infections, a common bacterial pathogen typically associated with meningitis in the adult and elderly population.^[2]
• Mycobacterium tuberculosis could also cause WFS. Tubercular invasion of the adrenal glands could cause hemorrhagic destruction of the glands and cause mineralocorticoid deficiency.
• Staphylococcus aureus has recently also been implicated in pediatric WFS.^[4]
• It can also be associated with Haemophilus influenzae.^[5][6]
• Cytomegalovirus can cause adrenal insufficiency,^[7] especially in the immunocompromised.

Prevention

Routine vaccination against meningococcus is recommended by the Centers for Disease Control for all 11–18 year olds and people who have poor splenic function (who, for example, have had their spleen removed or who have sickle-cell disease which damages the spleen), or who have certain immune disorders, such as a complement deficiency.^[8]

Treatment

Fulminant meningococcemia is a medical emergency and needs to be treated with adequate antibiotics as fast as possible. Benzylpenicillin was once the drug of choice with chloramphenicol as a good alternative in allergic patients. Ceftriaxone is an antibiotic commonly employed today. Hydrocortisone can sometimes reverse the hypoadrenal shock. Sometimes plastic surgery and grafting is needed to deal with tissue necrosis.

Historical

Waterhouse–Friderichsen syndrome is named after Rupert Waterhouse (1873–1958), an English physician, and Carl Friderichsen (1886–1979), a Danish pediatrician, who wrote papers on the syndrome, which had been previously described.^[9][10]

References

External links

• Whonamedit.com page on the eponym