The epidemiological and clinical characteristics of Cepacia syndrome, acute necrotizing pneumonia found in patients with cystic fibrosis (CF) years after initial infection with Burkholderia cepacia complex, were explored in a systematic review published in Pediatric Pulmonology. A high mortality rate was seen despite a range of treatments.

Upon searching MEDLINE, Embase, and the Cochrane Library databases, the study authors identified 15 eligible articles describing 18 cases of Cepacia syndrome; 9 patients were male and 9 were female. The patients had a median age at onset of 22 years and a median time of 5 years to Cepacia syndrome after the first infection by B Cepacia.

According to the reports, B cenocepacia was found in 11 patients and was the most common causative agent.

Moreover, most patients developed leukocytosis with a shift to the left. C-reactive protein (CRP) was elevated in all 5 studies that reported its levels, while blood cultures showed bacteremia in 11 out of 13 patients (84.6%). In the preterminal phases of the disease, bacteremia was present in 38% of cases.

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All patients were treated with intravenous antibiotics and were most frequently administered trimethoprim-sulfamethoxazole. Inhaled antibiotics were used to treat 5 patients (27.8%), while immunosuppressive agents were the most commonly prescribed supportive treatment (in 39% of patients).

Aligned with the high mortality associated with Cepacia syndrome, 50% of the reviewed patients (9 of 18) died.

“In the analyzed cases, quite different therapeutic options were used, both in terms of the type of antibiotics chosen for nebulization as well as in terms of supportive treatments associated with antimicrobial agents,” Daccò and colleagues noted.

“With regard to antibiotic treatment, our review suggests a positive effect of a combination of intensive IV and nebulized antibiotics regimen on the outcomes of CS, as this treatment was prescribed in 5/18 patients and all of them survived.”

Although there is limited information about the incidence of Cepacia syndrome, it is expected to have declined due to the strict patient segregation policy adopted by CF centers to minimize the spread of B Cepacia.


Daccò V, Alicandro G, Consales A, et al. Cepacia syndrome in cystic fibrosis: a systematic review of the literature and possible new perspectives in treatment. Published online February 23, 2023. Pediatr Pulmonol. doi:10.1002/ppul.26359