Waterhouse–Friderichsen syndrome

Waterhouse–Friderichsen syndrome
Other names: Hemorrhagic adrenalitis[1]
Top: Anatomically adrenal glands lie above the kidneys Bottom: Skin lesion(individual with WFS)
Specialty	Infectious disease, nephrology
Symptoms	Fever,joint pain,headache,vomiting[2]
Causes	Bacterial infection(Neisseria meningitidis)[2]
Diagnostic method	Clinical assessment, lab tests, and imaging[2]
Differential diagnosis	Septic shock and congenital adrenal hyperplasia due to 21-hydroxylase deficiency[2]
Treatment	Antibiotic, corticosteroids[2]

Waterhouse–Friderichsen syndrome (WFS) is defined as adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection. Typically, it is caused by Neisseria meningitidis.^[3][1]

The bacterial infection leads to massive bleeding into one or (usually) both adrenal glands.^[4] 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.^[5][1][2]

Ceftriaxone is an antibiotic commonly employed today for treatment^[6]

Signs and symptoms

Waterhouse-Friderichsen syndrome can be caused by a number of different organisms, when caused by Neisseria meningitidis, WFS is considered the most severe form of meningococcal sepsis. The onset of the illness is nonspecific with:^[1][2][5]

• Headache
• Vomit
• Fever
• Muscle pain
• Rash
• DIC
• Dizziness
• Hypotension

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.^[2][7]
• Pseudomonas aeruginosa can also cause WFS.^[1]
• WFS can also be caused by Streptococcus pneumoniae infections, a common bacterial pathogen typically associated with meningitis in the adult and elderly population.^[4]
• Mycobacterium tuberculosis could also cause WFS. Tubercular invasion of the adrenal glands could cause hemorrhagic destruction of the glands and cause mineralocorticoid deficiency^[8]

Mechanism

The pathogenesis of Waterhouse–Friderichsen syndrome is not clear^[2]

Diagnosis

Diagnostic criteria are based on clinical features of adrenal insufficiency as well as identifying the causal agent. If the causal agent is suspected to be meningitis a lumbar puncture is performed. If the causal agent is suspected to be bacterial a blood culture and complete blood count is performed. An adrenocorticotropic hormone stimulation test can be performed to assess adrenal function.^[1][2][5]

Differential diagnosis

In terms of the DDx we find the following should be considered:^[2]

• Septic shock
• Congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Prevention

Routine vaccination against meningococcus is recommended by the Centers for Disease Control and Prevention for all 11- to 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.^[9]

Treatment

Fulminant infection from meningococcal bacteria in the bloodstream is a medical emergency and requires emergent treatment with vasopressors, fluid resuscitation, and appropriate antibiotics. 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 adrenal insufficiency.^[2][1][5]

Prognosis

In terms of the prognosis we find that it depends on how severe the illness is; 15 percent with acute bilateral adrenal bleeding face a fatal outcome. If theres a delay in diagnosis and treatment, the fatality rate can be 50 percent^[2]

Epidemiology

In term of the incidence it is ~1 percent in post-mortem studies of patients with severe sepsis. Additionally, it is more common in children, with the highest incidence between 6 months and 1 year of age, and also reported during adolescence^[2][10]

History

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.^[11][12]

References

External links