European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis

Abstract

There is increasing awareness of bronchiectasis in children and adolescents, a chronic pulmonary disorder associated with poor quality-of-life for the child/adolescent and their parents, recurrent exacerbations and costs to the family and health systems. Optimal treatment improves clinical outcomes. Several national guidelines exist, but there are no international guidelines.The European Respiratory Society (ERS) Task Force for the management of paediatric bronchiectasis sought to identify evidence-based management (investigation and treatment) strategies. It used the ERS standardised process that included a systematic review of the literature and application of the GRADE approach to define the quality of the evidence and level of recommendations.A multidisciplinary team of specialists in paediatric and adult respiratory medicine, infectious disease, physiotherapy, primary care, nursing, radiology, immunology, methodology, patient advocacy and parents of children/adolescents with bronchiectasis considered the most relevant clinical questions (for both clinicians and patients) related to managing paediatric bronchiectasis. Fourteen key clinical questions (7 “Patient, Intervention, Comparison, Outcome” [PICO] and 7 narrative) were generated. The outcomes for each PICO were decided by voting by the panel and parent advisory group.This guideline addresses the definition, diagnostic approach and antibiotic treatment of exacerbations, pathogen eradication, long-term antibiotic therapy, asthma-type therapies (inhaled corticosteroids, bronchodilators), mucoactive drugs, airway clearance, investigation of underlying causes of bronchiectasis, disease monitoring, factors to consider before surgical treatment and the reversibility and prevention of bronchiectasis in children/adolescents. Benchmarking quality of care for children/adolescents with bronchiectasis to improve clinical outcomes and evidence gaps for future research could be based on these recommendations.

Topic Sessions

“…using high‐resolution computed tomography (HRCT) and better understanding of the underlying pathomechanisms, the vicious circle of infection, neutrophilic inflammation, impaired mucociliary clearance and airway wall destruction may be interrupted, existing changes reversed and irreversible changes prevented. It is therefore essential to promptly start a diagnostic process if chronic suppurative lung disease and bronchiectasis are suspected in a child or adolescent to identify treatable factors and start treatment as soon as possible (1). A likely precursor of pediatric BE is protracted bacterial bronchitis (PBB) which is defined as chronic productive cough which resolves with two weeks of appropriate antibiotic treatment.…”

“…(2)). General treatment recommendations for pediatric BE include regular application of airway clearance techniques, antibiotic treatment for exacerbations and consideration of long‐term macrolide treatment in those with frequent exacerbations (1).…”

Airway Clearance and Mucoactive Therapies

“…A lot of work still needs to be done, as is shown by recent data from a European audit that found that while approximately 45% of almost 5,800 patients with bronchiectasis performed ACT, adherence was low. 39 Until now ACTs have been strongly recommended by experts, but there is still a low level of evidence both in adults and children, 40 possibly due to the wide variety of techniques applied in the studies and also the diversity of outcomes studied in the articles.…”

Clinical and research priorities for children and young people with bronchiectasis: an international roadmap

“…Important biological differences include altered pathogen profiles (bacteria [ 8 ] and microbiota [ 9 ]), age-related immunological responses [ 10 ] and treatment outcomes [ 1 ]. Diagnostic [ 1 ] and treatment methods, such as airway clearance techniques (ACTs), may also differ according to age [ 11 , 12 ]. Additionally, comorbidities and underlying aetiologies are substantially different between adults and children [ 4 ].…”

“…To address the lack of published research priorities and patient-derived “needs assessment” data for CYP with bronchiectasis, we undertook this project as an extension of the European Respiratory Society (ERS) clinical practice guideline (CPG) for managing children and adolescents with bronchiectasis [ 12 ]. We sought to develop a roadmap to guide health services and research priorities pertinent to the needs of CYP (and their parents/carers) with bronchiectasis, in order to reduce their disease burden and improve their short- and long-term outcomes.…”