Variation in treatment strategies for the eradication of Pseudomonas aeruginosa in primary ciliary dyskinesia across European centers

Chronic Respiratory Disease

Published July 18 2018

Suzanne Crowley, Mathias Gelderman Holgersen, Kim Gjerum Nielsen

A common airway pathogen in PCD is Pseudomonas aeruginosa (Pa), and the prevalence of this bacteria increases with age. In the European PCD research consortium, Pa is estimated to affect 5% of children and 39% of adults, and those patients tend to have lower lung function and a delay in diagnosis. Recently, the European Respiratory Society (ERS) released guidelines for management of adult bronchiectasis — which includes PCD — stating that early detection, treatment, and eradication of Pa may improve clinical outcomes. However, a standard treatment protocol has not been established for PCD, which is why the European research group, BEAT-PCD, polled the European PCD centers about treatment regimen for patients with “acute, intermittent, and chronic Pa infection.”

Quick facts

  • Survey questionnaires were sent to 55 PCD centers in 36 European countries
  • Responses were received by 43 of those sites, a response rate of 70%
  • 87% of centers prescribed antibiotics for “newly acquired” Pa infection, 79% of which used inhaled antibiotics
  • 2 of the 3 adults centers only treated patients with Pa infection if/when lung function declined, while almost ¾ of pediatric sites treated Pa regardless of lung function
  • Breakdown of commonly prescribed treatments by center:
    •  Combination of oral ciprofloxacin and inhaled colistimethate sodium, prescribed in 18 (or 42%) of centers
    • Oral ciprofloxacin only, prescribed in 20% of centers
    • Inhaled tobramycin only, prescribed in 16% of centers
    • Combination of oral ciprofloxacin and inhaled tobramycin at 6 sites

Treatment for newly acquired Pseudomonas aeruginosa infection

  • Oral ciprofloxacin: 2 to 4 weeks
  • Inhaled antibiotics: 1 or 3 months (2 cycles)

Treatment for recurring Pseudomonas aeruginosa infection

  • If not eradicated after first course, IV antibiotics alone were used by 15% of sites, whereas a combination of IV antibiotics and inhaled antibiotics were used by 30% of sites
  • 10 days of IV antibiotic treatment was almost universally prescribed

Treatment for chronic infection of Pseudomonas aeruginosa 

  • Of the centers surveyed, 37 sites (86%) reported that the primary goal of treatment in chronic or colonized Pa infection was lung function “improvement and stabilization,” while eradication was deemed a tertiary endpoint
  • The most common approach for chronic infection is continuous inhaled antibiotics with oral ciprofloxacin
    • Inhaled colistimethate used in 22 PCD centers (51%)
    • Inhaled tobramycin used in 33% of PCD centers
    • Inhaled gentamycin used in just 6% of PCD centers
  • 25% of centers used inhaled antibiotics with IV antibiotics — carbapenem or ceftazidime
  • Another 25% of centers used inhaled antibiotics with a combination of two IV antibiotics — IV carbapenem or ceftazidime with IV tobramycin
  • Under 10% of sites used the combination of the 3 – inhaled antibiotics with 3 IV antibiotics

Most PCD centers participating in this study did not have guidelines for PCD-specific management, including the treatment of Pseudomonas aeruginosa. Consequently, there were no standard practices for frequency of patient visits, frequency of sputum cultures, medication options and duration of treatments. Nevertheless, because it is understood that Pa infection is associated with lung function deterioration, aggressive treatment is recommended in most cases. For newly acquired infection, the goal is to eradicate. With chronic infection, however, the aim is to preserve lung function and stabilize the patient. Clinical trials targeting Pa therapies are needed in the PCD population to help determine guidelines for treatment, but surveys like these are a great start to collecting clinical data.