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Nontuberculous mycobacterial (NTM) lung infections appear to be increasing in patients with cystic fibrosis (CF).
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Mycobacterium avium complex and Mycobacterium abscessus complex are the most frequently encountered NTM respiratory pathogens in patients with CF in most areas.
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Diagnosis of NTM lung disease in patients with CF generally follows American Thoracic Society and Cystic Fibrosis Foundation guidelines, with an emphasis on evaluating and treating all known comorbidities.
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Therapy for NTM in
Nontuberculous Mycobacterial Infections in Cystic Fibrosis
Section snippets
Key points
Epidemiology
The CF population has an especially high risk for NTM infection and poses unique challenges with regard to diagnosis, treatment, and prevention.4 Although the NTM isolation rate in the general population in North America ranges from approximately 6 to 22 per 100,000 and the NTM disease rate from 5 to 10 per 100,000,5 there is a 1000-fold greater prevalence of NTM in respiratory cultures from patients with CF. The reported prevalence of positive NTM cultures and/or NTM disease within various CF
Diagnosis of nontuberculous mycobacteria lung disease in cystic fibrosis
Given the ubiquitous nature of NTM, isolation of an NTM from a respiratory specimen is not synonymous with disease, nor is it necessarily an indication to initiate treatment. Current American Thoracic Society and Infectious Diseases Society of America (ATS/IDSA) criteria for the diagnosis of NTM lung disease calls for the presence of 2 or more positive cultures, in the setting of characteristic clinical symptoms and radiographic findings, and the exclusion of other diseases.4 These guidelines
Screening for nontuberculous mycobacteria in the cystic fibrosis population
NTM are often first detected in a CF sputum sample in the absence of clinical suspicion, as part of routine screening; the optimal frequency for such screening is not known. Among patients with CF 12 years of age or older in the US CF Patient Registry, 58% had mycobacterial cultures of which 14% were positive.12 Not all of these individuals had NTM lung disease, as small quantities of NTM from the environment may intermittently be present in the CF airway, but not result in NTM pulmonary
Treatment of nontuberculous mycobacteria lung disease
Treatment of NTM pulmonary disease in CF should be based on ATS/IDSA guidelines that were developed for the general population,4 as well as guidelines developed under the sponsorship of the US CF Foundation (CFF) and the European CF Society specific to individuals with CF.56
Nonpharmacologic treatment options
In addition to pharmacologic treatment of NTM infection, nonpharmacologic therapies for underlying CF lung disease that primarily target clearance of airway mucus obstruction are essential. All NTM treatment regimens need to be part of a comprehensive CF care plan that includes effective airway clearance, nutrition management, and treatment of CF comorbidities, such as sinus disease and CF-related diabetes. This care is most effectively delivered at a CF Care Center, which uses a
Surgical treatment options
Surgical resection (pneumonectomy, lobectomy, or segmentectomy) may be a consideration as adjuvant therapy to medical treatment of NTM pulmonary disease. Patients with MABSC lung disease but without CF have been reported to have a higher rate of sustained culture conversion after surgery than with antimicrobial therapy alone.91, 92 In patients with CF, often there is a lobe with a greater burden of disease; however, disease is generally diffuse and bronchiectasis eventually will involve all
Treatment outcomes and impact of nontuberculous mycobacteria lung disease
Treatment success is generally defined by sustained culture conversion for at least 12 months.4, 92 Rates of successful therapy appear to vary dramatically, based on NTM species, patterns of antibiotic resistance, and severity of disease, as seen in prospective trials in patients with NTM lung disease. In a recent retrospective review of patients with CF from Colorado, the rate of sustained culture conversion in response to initial treatment of MABSC (subspecies not designated) was 45%, and
Recommendations for follow-up
After a person with CF has been identified as having a positive NTM culture, close surveillance is warranted, with repeat sputum cultures obtained regularly.4 Additionally, people with CF with clinical decline or radiographic progression of lung disease that is unresponsive to treatment of typical CF pathogens should be evaluated for NTM. This is particularly important in patients previously infected with an NTM or treated for NTM pulmonary disease, as the presence of a second NTM is a
Lung transplantation in cystic fibrosis and nontuberculous mycobacteria
Bilateral lung transplantation is an option considered by patients with CF who develop severe bronchiectasis and end-stage lung disease, generally described as an FEV1 consistently below 30% predicted, a rapid decline in FEV1, or presence of increased frequency or severity of pulmonary exacerbations.96 In 2006, the International Society for Heart and Lung Transplantation included colonization with highly resistant or highly virulent bacteria, fungi, or mycobacteria in the list of relative
Infection prevention
Infections with NTM have historically been thought to be from environmental exposure8, 106; however, recently, the potential for patient-to-patient spread within CF centers has been described95, 107, 108, 109 with devastating consequences.109 Five patients who had overlapping clinical encounters at the University of Washington CF Center were found to have identical isolates of M abscessus subspecies massiliense by PFGE (pulsed-field gel electrophoresis) and repetitive unit-sequence–based
Summary/discussion
The prevalence of pulmonary NTM infections appears to be increasing in patients living with CF and, based on current trends, it seems likely that NTM infections will continue to increase. The steady improvement in survival achieved over the past 2 decades will likely lead to a greater proportion of the CF population resembling the phenotype best suited to MAC infection; those with less severe CFTR mutations and greater age. Likewise, it appears that the prevalence of MABSC is increasing
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2022, Journal of Cystic FibrosisCitation Excerpt :Nineteen epidemiological studies have been published to date, representing a total number of 23,418 CF-patients, 6% of whom were found to be infected with non-tuberculous mycobacteria (NTM) [1]. In fact, numbers vary from 3.3% and up to 13% from one study to another [2–9]. The risk factors for acquisition of NTM include concurrent chronic infection due to another infectious agent (Staphylococcus aureus, Aspergillus fumigatus, Stenotrophomonas maltophilia), older CF-patients, the use of oral corticosteroids and intravenous broad spectrum antimicrobial therapy [1,10].
This article originally appeared in Clinics in Chest Medicine, Volume 37, Issue 1, March 2016.
Disclosures: The authors are supported by the Cystic Fibrosis Foundation Research Development Grant (NICK15R0).