Waterhouse–Friderichsen syndrome

What causes Waterhouse-Friderichsen syndrome?

Waterhouse-Friderichsen syndrome is most often associated with meningococcal disease (accounts for 80% of cases). The syndrome also has been associated with other bacterial pathogens, including Streptococcus pneumoniae, group A beta-hemolytic streptococci, Neisseria gonorrhoeae, Escherichia coli , Klebsiella pneumoniae , Haemophilus influenzae (group B), Salmonella choleraesuis , Pasteurella multocida , Acinetobacter calcoaceticus , and Plesiomonas shigelloides. It may also be associated with a history of splenectomy. In rare cases, it may be caused by the use of medications that promote blood clotting, low platelet counts, primary antiphospholipid syndrome, renal vein thrombosis or steroid use. While the exact mechanism of disease is not clear, activation of several cytokine mediators appears to lead to sepsis and shock.

Last updated on 05-01-20

What is the prognosis for individuals with Waterhouse-Friderichsen syndrome?

In Waterhouse-Friderichsen syndrome, the case fatality rate is 55-60%, particularly when the diagnosis is delayed. Death typically results from sepsis, despite aggressive treatment.

Last updated on 05-01-20

How might Waterhouse-Friderichsen syndrome be treated?

Treatment may include antibiotics and glucocorticoids. Other treatment is symptomatic and supportive.

Last updated on 05-01-20

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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