Cepacia syndrome in cystic fibrosis: A systematic review of the literature and possible new perspectives in treatment

Abstract

Background: Cepacia syndrome (CS) is an acute, necrotizing pneumonia with a high mortality rate, occurring in patients with cystic fibrosis (CF) infected with Burkholderia cepacia complex (BCC). Due to its low incidence, data on this condition are limited.Methods: We conducted a systematic review of the reported cases of CS by searching MEDLINE, Embase and the Cochrane Library to improve knowledge of this rare but potentially lethal condition.Results: We included 15 eligible articles, describing 18 cases (9 females) of CS.Median age at onset was 22 years (range: 10-60 years); median time to CS after first infection by BCC was 5 years (range: 1-26 years). Burkholderia cenocepacia was the most frequently reported causative agent. All patients received intravenous antibiotic treatment (most frequently including cotrimoxazole), while inhaled antibiotics were used in five patients (27.8%). Immunosuppressant agents were the most commonly prescribed supportive treatment (n = 7, 38.9%). Half of the patients died (9/18, 50%).Conclusions: This study describes epidemiological, clinical characteristics, and prognosis of CS cases reported over the last 24 years. CS is a rare yet severe complication of BCC infection in patients with CF, which occurs several years after BCC colonization and has a negative outcome in 50% of the patients. Data are too scanty to identify the most effective therapeutic approach.

The microbiology and pathogenesis of nonfermenting Gram-negative infections

“…B. cepacia, B cenocepacia, B. multivorans, B. stabilis, B. vietnamiensis, B. solosa, B. Ambifaria, B. anthina, B. pyrrocinia, B. ubonensis ) that occur in several natural environments [79]. However, individual members of this complex can cause opportunistic nosocomial infections, especially in CF patients (as causative agent of the cepacian syndrome [80 ▪ ]) and immunocompromised individuals.…”

Current viewpoint on the epidemiology of nonfermenting Gram-negative bacterial strains

“…B. mallei is a rare zoonotic infection transmitted from infected solipeds [39]. Burkholderia cepacia complex has been associated with colonization and infection in patients with cystic fibrosis, and importantly, with the development of the Cepacia syndrome in this patient population, an acute necrotizing pneumonia that bears high mortality [40 ▪ ]. In addition, reports for several other uncommon NFGNB as cause of HCAIs have emerged in the recent years, mostly as case reports or case series [15 ▪▪ ,41,42 ▪ ,43 ▪ ].…”