Top research priorities with equal weight given to patient and clinician preferences, and separately for patients and clinicians
Overall rank | Patient rank | Clinician rank | Priority |
1 | 1 | 1 | Do corticosteroids or an alternative agent have the best risk–benefit to induce remission in adults with GLILD? |
2 | 2 | 4 | Do corticosteroids or an alternative agent have the best risk–benefit to maintain remission in GLILD? |
3 | 6 | 2 | In newly diagnosed GLILD, is first-line treatment superior to watchful waiting? |
4 | 4 | 5 | Are there specific risk factors in CVID for developing GLILD? |
5 | 3 | 8 | What is the optimal screening approach to detect incident cases of GLILD in people with CVID? |
6 | 9 | 6 | Are there specific pathological endotypes of GLILD with different natural history and treatment responses? |
7 | 13 | 3 | What is the value of a lung biopsy in the work-up of a patient with suspected GLILD? |
8 | 7 | 11 | What is the value of antifibrotic drugs such as pirfenidone and nintedanib in treating GLILD? |
=9 | 9 | 14 | Are there specific genetic endotypes of GLILD with different natural history and treatment response? |
=9 | 11 | 12 | Develop a discovery biomarker programme on blood and BAL to assist diagnosis and management of GLILD. |
11 | 4 | 20 | What is the benefit of a higher versus lower trough immunoglobulin replacement target in GLILD? |
12 | 11 | 15 | What is the value of CT-PET in the work-up of a patient with suspected GLILD? |
13 | 8 | 19 | Is GLILD a pathogen-driven local manifestation of a systemic immune dysregulation? |
=14 | 15 | 18 | Is immunosuppression for GLILD associated with increased risk of infection? |
=14 | 26 | 7 | What is the value of BAL in the work-up of a patient with suspected GLILD? |
16 | 18 | 16 | What is the value of blood or other biomarkers in the work-up of a patient with suspected GLILD? |
17 | 25 | 10 | Do higher or lower dose corticosteroids have the best risk–benefit to induce remission in GLILD? |
=18 | 13 | 23 | What is the role of B-cells in the pathogenesis of GLILD? |
=18 | 15 | 21 | Is GLILD an intrinsic dysregulation of the adaptive immune system? |
=18 | 20 | 16 | What is the optimal first-line treatment of GLILD in children? |
=18 | 23 | 13 | Which type of lung biopsy has the most favourable risk–benefit? |
=18 | 27 | 9 | What is the value of blood or other biomarkers in assessing disease activity? |
23 | 15 | 24 | What is the value of bone-marrow transplantation in the treatment of GLILD? |
=24 | 18 | 27 | Which epigenetic modifiers contribute to the manifestation of GLILD? |
=24 | 20 | 25 | What is the value of thoracic MRI in the work-up of a patient with suspected GLILD? |
=24 | 23 | 22 | What is the outcome of lung transplantation for GLILD? |
27 | 20 | 26 | Develop 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.