US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis

Abstract

Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease such as cystic fibrosis (CF). Pulmonary disease caused by NTM has emerged as a major threat to the health of individuals with CF but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened an expert panel of specialists to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM pulmonary disease in individuals with CF. Nineteen experts were invited to participate in the recommendation development process. Population, Intervention, Comparison, Outcome (PICO) methodology and systematic literature reviews were employed to inform draft recommendations. An anonymous voting process was used by the committee to reach consensus. All committee members were asked to rate each statement on a scale of: 0, completely disagree, to 9, completely agree; with 80% or more of scores between 7 and 9 being considered ‘good’ agreement. Additionally, the committee solicited feedback from the CF communities in the USA and Europe and considered the feedback in the development of the final recommendation statements. Three rounds of voting were conducted to achieve 80% consensus for each recommendation statement. Through this process, we have generated a series of pragmatic, evidence-based recommendations for the screening, investigation, diagnosis and treatment of NTM infection in individuals with CF as an initial step in optimising management for this challenging condition.

Changing Epidemiology of the Respiratory Bacteriology of Patients With Cystic Fibrosis

“…The CFF recommends at least annual screening for NTM, 20 but as evidenced in this study, many patients are screened several times per year, presumably because of providers' clinical concerns. The increased frequency of culturing may have contributed to the increased prevalence, although the frequency is still less than recommended by the CFF.…”

“…NTM cultures from sputum, induced sputum, or BAL from patients $ 12 years of age were analyzed, as younger children have inconsistent production of sputum, and throat swabs are unreliable to detect NTM. 20 Trends in incidence and prevalence were assessed by estimating the average, annual age-specific percent change, using Poisson regression models, accounting for repeated measures. Separate models were developed for each pathogen and parameter estimates were obtained for the whole population and stratified by age group.…”

Pulmonary Disease Caused by Non-Tuberculous Mycobacteria

“…Consensus recommendations for the management of NTM in individuals with cystic fibrosis have recently been published [16,17]. We review recent diagnostic innovations and advances in NTM-PD case management which are not specific to Europe alone.…”

Unusual discovery of a vestibular schwannoma following eradication therapy forMycobacterium abscessus

“…As median cystic fibrosis (CF) life expectancy rises, an increasing number of patients are becoming colonised with Mycobacterium abscessus 1; a rapidly growing non-tuberculous mycobacteria (NTM) ubiquitously found in the environment 1. NTM can cause progressive inflammatory lung damage, termed NTM-pulmonary disease (NTM-PD), or conversely, asymptomatic infection 1…”