TABLE 1

Top research priorities with equal weight given to patient and clinician preferences, and separately for patients and clinicians

Overall rankPatient rankClinician rankPriority
111Do corticosteroids or an alternative agent have the best risk–benefit to induce remission in adults with GLILD?
224Do corticosteroids or an alternative agent have the best risk–benefit to maintain remission in GLILD?
362In newly diagnosed GLILD, is first-line treatment superior to watchful waiting?
445Are there specific risk factors in CVID for developing GLILD?
538What is the optimal screening approach to detect incident cases of GLILD in people with CVID?
696Are there specific pathological endotypes of GLILD with different natural history and treatment responses?
7133What is the value of a lung biopsy in the work-up of a patient with suspected GLILD?
8711What is the value of antifibrotic drugs such as pirfenidone and nintedanib in treating GLILD?
=9914Are there specific genetic endotypes of GLILD with different natural history and treatment response?
=91112Develop a discovery biomarker programme on blood and BAL to assist diagnosis and management of GLILD.
11420What is the benefit of a higher versus lower trough immunoglobulin replacement target in GLILD?
121115What is the value of CT-PET in the work-up of a patient with suspected GLILD?
13819Is GLILD a pathogen-driven local manifestation of a systemic immune dysregulation?
=141518Is immunosuppression for GLILD associated with increased risk of infection?
=14267What is the value of BAL in the work-up of a patient with suspected GLILD?
161816What is the value of blood or other biomarkers in the work-up of a patient with suspected GLILD?
172510Do higher or lower dose corticosteroids have the best risk–benefit to induce remission in GLILD?
=181323What is the role of B-cells in the pathogenesis of GLILD?
=181521Is GLILD an intrinsic dysregulation of the adaptive immune system?
=182016What is the optimal first-line treatment of GLILD in children?
=182313Which type of lung biopsy has the most favourable risk–benefit?
=18279What is the value of blood or other biomarkers in assessing disease activity?
231524What is the value of bone-marrow transplantation in the treatment of GLILD?
=241827Which epigenetic modifiers contribute to the manifestation of GLILD?
=242025What is the value of thoracic MRI in the work-up of a patient with suspected GLILD?
=242322What is the outcome of lung transplantation for GLILD?
272026Develop a health-status questionnaire to assess burden in GLILD

GLILD: granulocytic–lymphocytic interstitial lung disease; CVID: common variable immunodeficiency disorders; BAL: bronchoalveolar lavage; CT: computed tomography; PET: positron emission tomography; MRI: magnetic resonance imaging.