Meningococcal Disease

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Basics

Description

  • Meningococcemia is a blood-borne infection caused by Neisseria meningitidis.
  • Bacteremia without meningitis: Patient is acutely ill and may have skin manifestations (rashes, petechiae, and ecchymosis) and hypotension.
  • Bacteremia with meningitis: sudden onset of fever, nausea, vomiting, headache, decreased ability to concentrate, and myalgias
  • Disease progresses rapidly (within hours).
  • Skin findings and hypotension may be present.
    • A petechial rash appears as discrete lesions 1 to 2 mm in diameter; most frequently on the trunk and lower portions of the body; seen in >50% of patients on presentation
    • Purpura fulminans is a severe complication of meningococcal disease and occurs in up to 25% of cases. It is characterized by acute onset of cutaneous hemorrhage and necrosis due to vascular thrombosis and disseminated intravascular coagulopathy.

Epidemiology

Incidence
  • The mortality rate is ~13%.
    • 11–19% of survivors suffer serious sequelae, including deafness, neurologic deficits, or limb loss due to peripheral ischemia.
  • Disease is seasonal, peaks in December/January.
  • In 2016, there were 375 cases of reported meningococcal disease (incidence rate of 0.18 cases per 100,000 persons) (1).
    • Most common in adolescents and young adults, followed by infants <1 year

Etiology and Pathophysiology

  • N. meningitidis is a gram-negative diplococcus with at least 13 serotypes.
  • N. meningitidis has an outer coat that produces disease-causing endotoxin.
  • Major serogroups in the United States are B, C, Y, and W-135.
    • Serogroup B is the predominant cause of meningococcemia in children <1 year.
    • Serogroup C is the most common cause of meningococcal disease in the United States.
    • Serogroup Y is the predominant cause of meningococcemia in the elderly (2).
  • Major serogroups worldwide are A, B, C, Y, and W-135.
    • W-135 is the major cause of disease in the “meningitis belt” of sub-Saharan Africa.

Genetics
Late complement component deficiency has an autosomal recessive inheritance.

Risk Factors

  • Age: 3 months to 1 year
  • Late complement component deficiency (C5, C6, C7, C8, or C9)
  • Asplenia (1)
  • Living in close quarters (e.g., household contacts, nursery/daycare, dormitories, military barracks)
  • Exposure to active (and/or) passive tobacco smoke (1)

General Prevention

  • Two vaccines are currently licensed for use in the United States. Each contains antigens to serogroups A, C, Y, and W-135. Neither provides immunity against serotype B, which is responsible for 1/3 of U.S. cases (3).
    • Meningococcal polysaccharide vaccine (MPSV-4): recommended for patients ≥55 years at elevated risk (1)
      • Short duration of protection: 1 to 3 years for patients age <5 years; 3 to 5 years for adolescents and adults (3)
      • Often used for patients requiring short duration of protection—traveling to endemic areas, college freshmen, community outbreaks (3)
    • Meningococcal conjugate vaccine (MCV-4): recommended for patients 2 to 55 years of age (1)
  • The FDA has licensed two serogroup B meningococcal (MenB) vaccines. The first (MenB-FHbp) is a 3-dose series. The second (MenB-4C) is a 2-dose series. Both vaccines were approved for use in persons aged 10 to 25 years. Individuals aged ≥10 years who are at increased risk for meningococcal disease due to persistent complement component deficiencies, anatomic or functional asplenia, should receive MenB vaccine (3).
  • Protective levels of antibody are achieved ~7 to 10 days after primary immunization (2).
  • Vaccine is recommended for everyone aged 11 to 18 years and individuals 19 to 55 years who are at increased risk for meningococcal disease.
    • Guillain-Barré syndrome has been associated with the MCV-4 vaccine; therefore, a personal history of Guillain-Barré is a relative contraindication for this vaccine.
  • CDC international travel advisory
    • Vaccine is required by the government of Saudi Arabia for Hajj pilgrims >2 years of age.
    • The vaccine should be given to travelers to sub-Saharan Africa (“meningitis belt”).

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