Report of the UK Cystic Fibrosis Trust Antibiotic Working Group

7. OTHER INFECTIONS7.1 Management of respiratory exacerbations in patients with Burkholderiacepacia complex7.1.1 IntroductionManagement of Burkholderia cepacia infection requires awareness of problems that may arise inculture and identification, including the consequences of recent taxonomic advances. 1–4 Briefly,isolates presently identified as ‘B.cepacia’ by conventional methods comprise several closely relatedbacterial species (sometimes referred to as genomovars) (table 7.1). Because of their phenotypicsimilarity they are collectively referred to as the B.cepacia complex (Bcc).Table 7.1: Taxonomy of the Burkholderia cepacia complex –genomovar status and species name.Genomovar SpeciesI Burkholderia cepaciaII Burkholderia multivoransIII Burkholderia cenocepaciaIV Burkholderia stabilisV Burkholderia vietnamiensisVI Burkholderia dolosaVII Burkholderia ambifariaVIII Burkholderia anthinaIX Burkholderia pyrrociniaX Burkholderia ubonensis? Burkholderia lateensBurkholderia diffusaBurkholderia arborisBurkholderia seminalisBurkholderia metallicaThe outcome of Bcc infection in patients with CF is variable. Some individuals experience frequentexacerbations of their pulmonary disease, similar to those seen in patients with chronic P.aeruginosainfection; others have no symptoms or succumb to the rapidly fatal pneumonia known as ‘cepaciasyndrome’. 5–8 Some members of the Bcc are more closely associated with ‘cepacia syndrome’ andpatient-to-patient spread, in particular Burkholderia cenocepacia. 9–11 Other species such asBurkholderia multivorans 12;13 have also been associated with ‘cepacia syndrome’ and some, such asBurkholderia dolosa appear as invasive in vitro as B.cenocepacia. 14 Chronic infection with B.dolosa hasalso been associated with accelerated decline in lung function in patients with CF. 15Studies suggest that the epidemiology of Bcc has changed in recent years in CF units. Successfulsegregation policies have resulted in a decline in the prevalence of B.cenocepacia and in manyEuropean CF centres the most common Bcc species is now B.multivorans. 16;17 [3] Even in countrieswhere B.cenocepacia remains the predominant species, such as the USA, most recent acquisitions havebeen with B.multivorans. 18 [3] Genotyping evidence also suggests that most isolates of B.multivoransappear largely unrelated between different patients, suggesting possible acquisition from theenvironment rather than from other patients with CF. 19 [3] Isolates of Bcc can be found in a varietyof environmental niches such as soil and water, but exactly how patients with CF acquire manymembers of the Bcc such as B.multivorans remains uncertain. 20 [3]Cystic Fibrosis Trust 7.0March 2009