Waterhouse–Friderichsen syndrome

Life-threatening adrenal hemorrhage and adrenal crisis classically associated with fulminant meningococcemia

Waterhouse–Friderichsen syndrome
Synonyms	Waterhouse-Friderichsen syndrome, WFS, hemorrhagic adrenalitis, adrenal apoplexy, acute adrenal hemorrhage with adrenal insufficiency
Pronounce	N/A
Specialty	Emergency medicine, Critical care medicine, Endocrinology, Infectious disease, Pediatrics
Symptoms	Fever, purpura, petechiae, hypotension, shock, vomiting, altered mental status, abdominal pain, weakness
Complications	Septic shock, adrenal crisis, disseminated intravascular coagulation, multi-organ failure, limb ischemia, coma, death
Onset	Sudden or rapidly progressive
Duration	Acute medical emergency
Types	N/A
Causes	Bilateral adrenal hemorrhage, classically from severe Neisseria meningitidis infection
Risks	Meningococcemia, sepsis, asplenia, complement deficiency, complement inhibitor therapy, anticoagulation, severe physiologic stress, trauma, pregnancy, antiphospholipid syndrome
Diagnosis	Clinical suspicion, sepsis evaluation, serum cortisol and ACTH when possible, blood cultures, coagulation studies, electrolytes, CT or MRI evidence of adrenal hemorrhage
Differential diagnosis	Septic shock, meningococcemia, adrenal crisis, purpura fulminans, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, anaphylaxis
Prevention	Meningococcal vaccine, prompt treatment of sepsis, chemoprophylaxis for close contacts of meningococcal disease, risk-based vaccination
Treatment	Immediate antibiotics, aggressive fluid resuscitation, vasopressors, stress-dose hydrocortisone, intensive care support, correction of coagulopathy
Medication	Ceftriaxone, cefotaxime, broad-spectrum antibiotics, hydrocortisone, vasopressors
Prognosis	Poor without rapid treatment; mortality remains high in fulminant cases
Frequency	Rare
Deaths	N/A

Waterhouse–Friderichsen syndrome (WFS) is a rare, rapidly progressive, and life-threatening condition characterized by acute adrenal gland hemorrhage with adrenal insufficiency or adrenal crisis, most classically in the setting of fulminant meningococcemia caused by Neisseria meningitidis. It can also occur with other severe infections, coagulopathy, anticoagulant therapy, thrombotic disorders, trauma, or extreme physiologic stress.Waterhouse-Friderichsen Syndrome(link). StatPearls, NCBI Bookshelf.

The syndrome is a medical emergency. Patients may develop fever, vomiting, rapidly spreading petechiae or purpura, hypotension, septic shock, disseminated intravascular coagulation, electrolyte abnormalities, acute adrenal failure, coma, and death. Treatment requires immediate recognition, broad empiric antibiotics, aggressive resuscitation, intensive care, vasopressors when needed, and stress-dose hydrocortisone for suspected adrenal crisis.Clinical Guidance for Meningococcal Disease(link). Centers for Disease Control and Prevention."Guidance for the prevention and emergency management of adult patients with adrenal insufficiency".Clinical Medicine.2020;PMC:7385786.

Overview

Waterhouse–Friderichsen syndrome is best understood as the intersection of severe systemic illness, adrenal hemorrhage, and acute adrenal failure. The classic presentation is fulminant meningococcal sepsis with purpura fulminans and bilateral adrenal hemorrhage. However, modern case reports and reviews show that the syndrome is not limited to meningococcal disease and can occur in adults or children with other infections or noninfectious causes of adrenal bleeding.Waterhouse-Friderichsen Syndrome(link). StatPearls, NCBI Bookshelf."Waterhouse-Friderichsen Syndrome with Bilateral Adrenal Hemorrhage Secondary to Methicillin-Resistant Staphylococcus aureus Bacteremia".American Journal of Case Reports.2022;PMC:9016783.

The adrenal glands are highly vascular endocrine organs. During severe sepsis, shock, coagulopathy, or thrombosis, adrenal venous drainage can be overwhelmed, predisposing to hemorrhage. When both adrenal glands are affected, cortisol production may abruptly fall, worsening hypotension and shock.

Terminology

• Waterhouse–Friderichsen syndrome - Acute adrenal hemorrhage with adrenal insufficiency, classically associated with meningococcemia.
• Hemorrhagic adrenalitis - Bleeding and inflammation of the adrenal glands.
• Adrenal apoplexy - Sudden adrenal hemorrhage.
• Bilateral adrenal hemorrhage - Bleeding into both adrenal glands.
• Adrenal crisis - Life-threatening acute cortisol deficiency with hypotension or shock.
• Meningococcemia - Bloodstream infection with Neisseria meningitidis.
• Purpura fulminans - Severe thrombotic and hemorrhagic skin disorder often associated with DIC and sepsis.
• Septic shock - Sepsis with circulatory and metabolic abnormalities causing high mortality.
• Disseminated intravascular coagulation - Systemic activation of clotting with bleeding and thrombosis.
• Adrenocortical insufficiency - Inadequate adrenal cortical hormone production.

Anatomy and physiology

The adrenal glands sit above the kidneys and contain two major regions.

• Adrenal cortex - Produces cortisol, aldosterone, and adrenal androgens.
• Adrenal medulla - Produces catecholamines such as epinephrine and norepinephrine.
• Cortisol - Essential glucocorticoid hormone for stress response, blood pressure maintenance, glucose metabolism, and immune regulation.
• Aldosterone - Mineralocorticoid hormone that helps regulate sodium, potassium, and blood pressure.
• Adrenocorticotropic hormone - Pituitary hormone that stimulates cortisol production.
• Hypothalamic-pituitary-adrenal axis - Hormonal stress-response system.

In Waterhouse–Friderichsen syndrome, adrenal hemorrhage can destroy adrenal cortical tissue or impair adrenal function, causing sudden cortisol deficiency during an already severe illness.

Causes

Meningococcal infection

The classic cause of WFS is fulminant infection with Neisseria meningitidis.

• Neisseria meningitidis - Gram-negative diplococcus that can cause meningitis, meningococcemia, sepsis, and purpura fulminans.
• Meningococcemia - Bloodstream infection that can progress rapidly.
• Meningococcal meningitis - Meningitis may occur with meningococcemia but is not always present in WFS.
• Endotoxin - Meningococcal lipooligosaccharide contributes to inflammatory shock.
• Purpura fulminans - Severe manifestation of meningococcal sepsis.
• Complement deficiency - Increases risk of invasive meningococcal disease.
• Asplenia - Increases risk of severe infection with encapsulated bacteria.

Other bacterial causes

Many bacteria can rarely cause adrenal hemorrhage or WFS-like adrenal failure.

• Streptococcus pneumoniae
• Staphylococcus aureus, including methicillin-resistant Staphylococcus aureus
• Haemophilus influenzae
• Pseudomonas aeruginosa
• Escherichia coli
• Klebsiella pneumoniae
• Capnocytophaga canimorsus
• Group A Streptococcus
• Mycobacterium tuberculosis
• Rickettsial infection
• Septicemia

Viral, fungal, and opportunistic causes

• Cytomegalovirus - Can involve the adrenal glands, especially in immunocompromised patients.
• Ebola virus disease - Severe viral hemorrhagic fever may involve adrenal dysfunction.
• HIV/AIDS - Can predispose to opportunistic adrenal infection or adrenal insufficiency.
• Fungal infection - Disseminated fungal disease may involve adrenal glands.
• Histoplasmosis - Can cause adrenal enlargement and adrenal insufficiency.
• Candidemia - Rarely associated with adrenal involvement.

Noninfectious causes

Waterhouse–Friderichsen syndrome is classically infectious, but bilateral adrenal hemorrhage with acute adrenal crisis can also occur without meningococcemia.

• Anticoagulant therapy - Warfarin, heparin, and direct oral anticoagulants may increase bleeding risk.
• Antiphospholipid syndrome - Thrombotic disorder associated with adrenal hemorrhage.
• Heparin-induced thrombocytopenia - Can cause adrenal vein thrombosis and hemorrhage.
• Trauma - Blunt abdominal trauma can cause adrenal hemorrhage.
• Major surgery - Severe postoperative stress may be associated with adrenal hemorrhage.
• Burn injury - Severe physiologic stress can predispose to adrenal hemorrhage.
• Pregnancy - Rare cases occur during pregnancy or postpartum.
• Severe stress - Critical illness may contribute to adrenal bleeding.
• Adrenal tumor - Hemorrhage into an adrenal mass can mimic or contribute to adrenal crisis.
• Coagulopathy - Bleeding disorders increase risk.
• Thrombocytopenia - Low platelet count increases bleeding risk.

Risk factors

• Meningococcal disease
• Asplenia
• Sickle cell disease
• Complement deficiency
• Complement inhibitor therapy, such as eculizumab or ravulizumab
• Immunocompromised state
• Sepsis
• Disseminated intravascular coagulation
• Anticoagulation
• Antiphospholipid syndrome
• Heparin-induced thrombocytopenia
• Pregnancy
• Trauma
• Adrenal mass
• Critical illness
• Lack of age-appropriate meningococcal vaccine

CDC notes that people receiving complement inhibitors remain at substantial risk for meningococcal disease even with vaccination, and additional prophylaxis may be considered in selected patients.Clinical Guidance for Managing Meningococcal Disease Risk in Patients Receiving Complement Inhibitor Therapy(link). Centers for Disease Control and Prevention.

Pathophysiology

The syndrome develops through several overlapping mechanisms.

• Sepsis - Systemic infection causes inflammation, vasodilation, capillary leak, and organ dysfunction.
• Endotoxemia - Bacterial toxins trigger inflammatory and coagulation pathways.
• Adrenal hemorrhage - Bleeding into adrenal tissue impairs hormone production.
• Adrenal vein thrombosis - Venous outflow obstruction may cause hemorrhagic infarction.
• Disseminated intravascular coagulation - Consumes platelets and clotting factors, causing bleeding and microthrombosis.
• Purpura fulminans - Skin necrosis and purpura due to DIC and vascular thrombosis.
• Cortisol deficiency - Worsens shock, hypoglycemia, hyponatremia, and vascular instability.
• Mineralocorticoid deficiency - Can contribute to hyponatremia, hyperkalemia, and hypotension.
• Multi-organ failure - Shock and microvascular thrombosis damage kidneys, lungs, brain, liver, and extremities.

The combination of infection, inflammation, coagulation activation, adrenal bleeding, and cortisol deficiency creates a rapidly worsening shock state.

Signs and symptoms

Symptoms often begin nonspecifically and then progress quickly.

Early symptoms

• Fever
• Chills
• Rigors
• Headache
• Nausea
• Vomiting
• Abdominal pain
• Back pain
• Myalgia
• Fatigue
• Malaise
• Dizziness
• Confusion
• Irritability in children
• Poor feeding in infants

Severe symptoms

• Petechiae - Small nonblanching red or purple spots.
• Purpura - Larger purple skin lesions caused by bleeding into skin.
• Purpura fulminans - Rapidly progressive purpura with skin necrosis and shock.
• Hypotension - Low blood pressure.
• Septic shock - Severe infection with life-threatening circulatory failure.
• Cyanosis - Bluish discoloration due to poor oxygenation or perfusion.
• Cold extremities
• Tachycardia
• Tachypnea
• Altered mental status
• Coma
• Oliguria - Decreased urine output.
• Acute kidney injury
• Respiratory failure
• Multi-organ failure

Features of adrenal crisis

• Hypotension refractory to fluids
• Shock
• Hypoglycemia
• Hyponatremia
• Hyperkalemia
• Metabolic acidosis
• Severe weakness
• Vomiting
• Abdominal pain
• Confusion
• Collapse

Not every patient has the full classic picture. Absence of meningitis, absence of extreme leukocytosis, or initially subtle rash does not exclude WFS.

Diagnosis

Diagnosis is clinical and should not delay treatment. Waterhouse–Friderichsen syndrome should be suspected in a patient with sepsis, shock, purpura, DIC, and signs of adrenal insufficiency, especially when meningococcemia is possible.

Emergency evaluation

• Vital signs - Blood pressure, pulse, respiratory rate, oxygen saturation, temperature.
• Sepsis evaluation - Rapid assessment for infection and organ dysfunction.
• Blood culture - Should be obtained promptly if possible before antibiotics, but antibiotics should not be delayed.
• Complete blood count - May show leukocytosis, leukopenia, anemia, or thrombocytopenia.
• Comprehensive metabolic panel - Evaluates sodium, potassium, glucose, kidney function, and liver function.
• Serum cortisol - May support diagnosis but should not delay hydrocortisone in adrenal crisis.
• Adrenocorticotropic hormone - Can help distinguish primary adrenal failure when collected before steroids, if feasible.
• Coagulation studies - PT, INR, aPTT, fibrinogen, and D-dimer evaluate DIC.
• Lactate - Marker of shock and tissue hypoperfusion.
• Arterial blood gas or venous blood gas - Assesses acidosis and respiratory status.
• C-reactive protein and procalcitonin - May support infection assessment but are not definitive.
• Urinalysis - Evaluates kidney and infection clues.
• Lumbar puncture - May be performed when meningitis is suspected and patient is stable, but should not delay antibiotics.
• Skin lesion culture or microscopy - May identify meningococci in purpuric lesions in some cases.

Imaging

Imaging may confirm adrenal hemorrhage but must not delay resuscitation in unstable patients.

• Computed tomography - CT abdomen is commonly used to identify adrenal hemorrhage.
• Magnetic resonance imaging - Can characterize adrenal hemorrhage, especially when diagnosis is uncertain.
• Ultrasound - May be useful in neonates or unstable patients, but is less sensitive than CT.
• Chest radiograph - Assesses pneumonia, respiratory failure, or line placement.
• Point-of-care ultrasound - May help assess shock and volume status.
• Adrenal enlargement - Imaging sign of acute hemorrhage.
• Periadrenal stranding - CT feature that may accompany hemorrhage.

A 2022 imaging review described urgent CT as a key diagnostic test when adrenal hemorrhage is suspected, while recognizing that unstable patients require immediate clinical treatment."Multimodality Imaging Findings in Waterhouse-Friderichsen Syndrome".Journal of Imaging.2022;8(12)

330.doi:10.3390/jimaging8120330.

Microbiologic diagnosis

• Blood culture - Important for meningococcemia and other bacteremia.
• Cerebrospinal fluid culture - Used when meningitis is suspected.
• Polymerase chain reaction - Can detect meningococcal DNA in some settings.
• Gram stain - May show gram-negative diplococci in meningococcal disease.
• Antimicrobial susceptibility testing - Guides therapy and prophylaxis decisions.
• Respiratory culture - May identify pneumonia source.
• Urine culture - May identify urinary sepsis.
• Molecular testing - May identify pathogens when cultures are negative after antibiotics.

Differential diagnosis

• Septic shock
• Meningococcemia
• Adrenal crisis
• Bilateral adrenal hemorrhage
• Purpura fulminans
• Disseminated intravascular coagulation
• Rocky Mountain spotted fever
• Toxic shock syndrome
• Staphylococcal scalded skin syndrome
• Severe sepsis
• Anaphylaxis
• Acute hemorrhage
• Thrombotic thrombocytopenic purpura
• Hemolytic uremic syndrome
• Immune thrombocytopenia
• Leukemia
• Antiphospholipid syndrome
• Heparin-induced thrombocytopenia
• Acute abdomen
• Ruptured abdominal aortic aneurysm
• Adrenal infarction
• Adrenal tumor hemorrhage

Treatment

Treatment must begin immediately when WFS or fulminant meningococcemia is suspected. Management occurs in an emergency department or intensive care unit.

Immediate management

• Airway management - Secure airway if mental status or respiratory failure requires it.
• Oxygen therapy - Treat hypoxemia.
• Intravenous access - Large-bore access or central venous access may be needed.
• Fluid resuscitation - Rapid isotonic crystalloid resuscitation for shock.
• Vasopressor - Norepinephrine is commonly used when shock persists after fluids.
• Broad-spectrum antibiotics - Give promptly for suspected bacterial sepsis.
• Ceftriaxone or cefotaxime - Recommended empiric therapy for suspected meningococcal disease.
• Hydrocortisone - Give stress-dose steroid for suspected adrenal crisis.
• Glucose - Treat hypoglycemia immediately.
• Electrolyte correction - Correct severe hyperkalemia, hyponatremia, and acidosis.
• Blood product support - Platelets, plasma, cryoprecipitate, or red cells may be needed for bleeding or DIC.
• Intensive care unit - Required for shock, organ failure, or respiratory failure.

CDC clinical guidance states that suspected meningococcal disease requires prompt antibiotics and that empiric therapy should include an extended-spectrum cephalosporin such as cefotaxime or ceftriaxone.Clinical Guidance for Meningococcal Disease(link). Centers for Disease Control and Prevention.

Adrenal crisis treatment

When adrenal crisis is suspected, hydrocortisone should be given immediately rather than waiting for confirmatory testing.

• Hydrocortisone - Emergency glucocorticoid replacement.
• Stress dose - High-dose glucocorticoid dosing used during severe illness.
• Normal saline - Fluid resuscitation for hypotension and volume depletion.
• Dextrose - Used when hypoglycemia is present or likely.
• Fludrocortisone - Usually not needed acutely when high-dose hydrocortisone is used, but may be needed later in primary adrenal insufficiency.
• Cortisol testing - Draw blood before steroid only if it does not delay treatment.
• ACTH stimulation test - May be performed later when the patient is stable.

Emergency adrenal insufficiency guidance recommends prompt hydrocortisone for suspected adrenal crisis, commonly 100 mg intravenously or intramuscularly followed by ongoing hydrocortisone dosing."Guidance for the prevention and emergency management of adult patients with adrenal insufficiency".Clinical Medicine.2020;PMC:7385786.Primary Adrenal Insufficiency Guideline Resources(link). Endocrine Society.

Antibiotic therapy

Antibiotic choice depends on suspected source, age, local resistance, immune status, and culture results.

• Ceftriaxone - Common empiric treatment for suspected meningococcal disease.
• Cefotaxime - Alternative extended-spectrum cephalosporin.
• Vancomycin - Added when resistant gram-positive infection or bacterial meningitis differential requires it.
• Ampicillin - Added in some meningitis regimens when Listeria monocytogenes is a concern.
• Meropenem - Used in selected severe sepsis or resistant organism concerns.
• Penicillin G - May be used for susceptible meningococcal disease after confirmation.
• Antimicrobial susceptibility testing - Helps narrow treatment.
• Source control - Drainage or surgery may be needed for abscess or infected tissue.

Management of DIC and purpura fulminans

• Disseminated intravascular coagulation treatment - Treat underlying sepsis and support coagulation.
• Platelet transfusion - Used for severe thrombocytopenia with bleeding or procedures.
• Fresh frozen plasma - Used for bleeding with coagulation factor deficiency.
• Cryoprecipitate - Used for low fibrinogen with bleeding.
• Heparin - Sometimes considered in selected thrombotic purpura fulminans but requires specialist input.
• Wound care - Necessary for skin necrosis.
• Plastic surgery - May be needed for reconstructive care.
• Amputation - Sometimes required for irreversible limb ischemia or necrosis.

Supportive and long-term care

• Mechanical ventilation - For respiratory failure.
• Renal replacement therapy - For severe acute kidney injury.
• Nutrition support - Needed during critical illness recovery.
• Rehabilitation - After limb ischemia, amputation, weakness, or neurologic injury.
• Endocrinology follow-up - Evaluates long-term adrenal function.
• Infectious disease consultation - Guides antimicrobial therapy and public health measures.
• Psychological support - Important after critical illness, limb loss, or trauma.
• Vaccination review - Update meningococcal and other vaccines when stable.

Prevention

Prevention focuses on reducing invasive meningococcal disease and preventing severe infection in high-risk groups.

Meningococcal vaccination

• MenACWY vaccine - Protects against meningococcal serogroups A, C, W, and Y.
• MenB vaccine - Protects against serogroup B meningococcal disease.
• Adolescent vaccination - Routine meningococcal vaccination is recommended in many countries.
• College student vaccination - Important for students in dormitory settings.
• Asplenia - Requires meningococcal vaccination because risk is increased.
• Complement deficiency - Requires risk-based meningococcal vaccination.
• Complement inhibitor therapy - Requires vaccination and risk counseling.
• Travel vaccination - Needed for some travelers to areas with meningococcal disease risk.
• Booster dose - Needed in some groups with ongoing risk.

CDC provides current recommendations for meningococcal vaccination by age and risk group, including adolescents and people with increased risk such as asplenia, complement deficiency, or complement inhibitor therapy.Meningococcal Vaccine Recommendations(link). Centers for Disease Control and Prevention.

Chemoprophylaxis for contacts

Close contacts of invasive meningococcal disease patients require prompt antibiotic prophylaxis.

• Rifampin
• Ciprofloxacin
• Ceftriaxone
• Azithromycin in selected resistance situations
• Household contacts
• Childcare contacts
• Direct exposure to oral secretions
• Healthcare exposure during airway management without proper protection

CDC recommends antibiotic prophylaxis for close contacts of meningococcal disease patients, ideally as soon as possible after exposure.Public Health Strategies for Antibiotic-resistant Neisseria meningitidis(link). Centers for Disease Control and Prevention.Meningococcal Disease(link). CDC Yellow Book, NCBI Bookshelf.

General prevention of severe infection

• Sepsis awareness
• Early treatment of fever and rash
• Vaccination against encapsulated bacteria in asplenia
• Prompt care for immunocompromised patients
• Safe anticoagulant monitoring
• Infection prevention in healthcare settings
• Patient education for complement inhibitor recipients
• Medical alert identification for adrenal insufficiency when present

Complications

• Adrenal crisis
• Septic shock
• Disseminated intravascular coagulation
• Purpura fulminans
• Acute kidney injury
• Acute respiratory distress syndrome
• Liver dysfunction
• Myocardial dysfunction
• Hypoglycemia
• Hyponatremia
• Hyperkalemia
• Metabolic acidosis
• Coma
• Limb ischemia
• Skin necrosis
• Amputation
• Hearing loss after meningococcal disease
• Neurologic injury
• Post-intensive care syndrome
• Death

Prognosis

The prognosis depends on the cause, speed of recognition, timing of antibiotics, severity of shock, degree of adrenal destruction, extent of DIC, and presence of multi-organ failure. Fulminant meningococcemia with WFS can be fatal within hours if not recognized and treated quickly.

• Early antibiotics improve survival in meningococcal disease.
• Hydrocortisone can be lifesaving when adrenal crisis contributes to shock.
• Severe purpura fulminans increases risk of amputation and death.
• Survivors may require rehabilitation after critical illness, skin necrosis, or limb loss.
• Long-term adrenal function should be reassessed after recovery.
• Some patients may require temporary or permanent glucocorticoid and mineralocorticoid replacement.

Epidemiology

Waterhouse–Friderichsen syndrome is rare. It is most often described as a complication of fulminant meningococcal sepsis but has been reported with many other pathogens and noninfectious conditions. Its frequency has decreased in settings with effective meningococcal vaccination, rapid antibiotics, and improved intensive care, but it remains a critical emergency because of its rapid progression and high mortality.

• Children and adolescents can be affected by meningococcal WFS.
• Adults can develop WFS from meningococcal or nonmeningococcal sepsis.
• Asplenic and complement-deficient patients are at increased risk for invasive meningococcal disease.
• Complement inhibitor recipients are at markedly increased risk of meningococcal disease.
• WFS may be underrecognized in noninfectious bilateral adrenal hemorrhage.

History

Waterhouse–Friderichsen syndrome is named for Rupert Waterhouse, an English physician, and Carl Friderichsen, a Danish pediatrician, who described cases of adrenal hemorrhage associated with severe infection in the early 20th century. Earlier descriptions of adrenal apoplexy also existed before the eponym became widely used.

• Rupert Waterhouse - Described a case of suprarenal apoplexy in 1911.
• Carl Friderichsen - Described adrenal apoplexy in children in 1918.
• Suprarenal apoplexy - Historical term for adrenal hemorrhage.
• Meningococcemia - Later recognized as the classic infectious association.
• Adrenal steroid therapy - Became part of management once adrenal failure was understood.

Patient education

Waterhouse–Friderichsen syndrome is not a condition that can be managed at home. It is an emergency requiring hospital and intensive care treatment.

• Fever with a rapidly spreading purple rash is an emergency.
• Confusion, fainting, low blood pressure, or cold extremities with fever requires urgent care.
• Meningococcal disease can progress very rapidly.
• Vaccination reduces risk of meningococcal disease.
• Close contacts of meningococcal disease patients may need preventive antibiotics.
• People without a spleen should stay current on vaccines.
• People taking complement inhibitors should understand their meningococcal risk.
• Survivors may need endocrine follow-up for adrenal function.
• Medical alert identification may be needed if long-term adrenal insufficiency remains.

When to seek emergency care

Immediate emergency care is required for symptoms suggesting meningococcemia, sepsis, or adrenal crisis.

• Fever with petechiae or purpura
• Fever with severe weakness or collapse
• Hypotension
• Confusion
• Coma
• Severe headache with fever
• Neck stiffness with fever
• Seizure
• Rapid breathing
• Cold, blue, or mottled extremities
• Severe abdominal or back pain with shock
• Repeated vomiting with weakness
• Signs of septic shock
• Known adrenal insufficiency with severe illness
• Recent close exposure to meningococcal disease

See also

• Adrenal gland
• Adrenal hemorrhage
• Adrenal crisis
• Adrenal insufficiency
• Meningococcemia
• Neisseria meningitidis
• Meningococcal disease
• Meningococcal vaccine
• Septic shock
• Sepsis
• Purpura fulminans
• Disseminated intravascular coagulation
• Asplenia
• Complement deficiency
• Hydrocortisone
• Ceftriaxone
• Cefotaxime
• Critical care medicine
• Emergency medicine
• Endocrinology
• Infectious disease

Further reading

• Waterhouse-Friderichsen Syndrome(link). StatPearls, NCBI Bookshelf.
• Clinical Guidance for Meningococcal Disease(link). Centers for Disease Control and Prevention.
• Meningococcal Vaccine Recommendations(link). Centers for Disease Control and Prevention.
• Clinical Guidance for Managing Meningococcal Disease Risk in Patients Receiving Complement Inhibitor Therapy(link). Centers for Disease Control and Prevention.
• Meningococcal Disease(link). CDC Yellow Book, NCBI Bookshelf.
• Primary Adrenal Insufficiency Guideline Resources(link). Endocrine Society.
• "Guidance for the prevention and emergency management of adult patients with adrenal insufficiency".Clinical Medicine.2020;PMC:7385786.
• "Waterhouse-Friderichsen Syndrome with Bilateral Adrenal Hemorrhage Secondary to Methicillin-Resistant Staphylococcus aureus Bacteremia".American Journal of Case Reports.2022;PMC:9016783.
• "Fatal Waterhouse-Friderichsen syndrome in an adult due to Streptococcus pneumoniae infection".IDCases.2021;PMC:7868193.
• "Multimodality Imaging Findings in Waterhouse-Friderichsen Syndrome".Journal of Imaging.2022;8(12)

330.doi:10.3390/jimaging8120330.

External links

• NCBI Bookshelf - Waterhouse-Friderichsen Syndrome
• CDC - Clinical Guidance for Meningococcal Disease
• CDC - Meningococcal Vaccine Recommendations
• CDC - Meningococcal disease risk and complement inhibitors
• CDC Yellow Book - Meningococcal Disease
• Endocrine Society - Primary Adrenal Insufficiency Guideline
• Guidance for emergency management of adrenal insufficiency
• MRSA bacteremia and Waterhouse-Friderichsen syndrome
• Pneumococcal Waterhouse-Friderichsen syndrome
• Multimodality imaging findings in Waterhouse-Friderichsen syndrome

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