Burkholderia cepacia and related species (B cepacia complex, BCC) can result in fulminant disease that is usually fatal, termed the "Cepacia Syndrome".
The typical patient has cystic fibrosis and may have had asymptomatic carriage for years.
Clinical features:
(1) high fevers
(2) rapidly progressive pneumonia
(3) bacteremia
(4) rapid decline in respiratory function progressing to respiratory failure
(5) death
Chest X-rays show an infiltrate that rapidly becomes confluent.
Histology of the pneumonia shows widespread necrotizing granulomas.
Laboratory findings:
(1) marked leukocytosis
(2) positive sputum and blood cultures
(3) worsening hypoxemia
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