Granulomatous–lymphocytic interstitial lung disease: an international research prioritisation

John R. Hurst; S. Hamza Abbas; Heba M. Bintalib; Tiago M. Alfaro; Ulrich Baumann; Siobhan O. Burns; Alison Condliffe; Jesper R. Davidsen; Børre Fevang; Andrew R. Gennery; Filomeen Haerynck; Joseph Jacob; Stephen Jolles; Olivia Lamers; Anne Bergeron; Marion Malphettes; Véronique Meignin; Cinzia Milito; Tomas Milota; Martine Pergent; Antje Prasse; Isabella Quinti; Elisabetta Renzoni; Anna Sediva; Daiana Stolz; Bas Smits; Friedolin Strauss; Annick A.J.M. van de Ven; Joris van Montfrans; Klaus Warnatz

doi:10.1183/23120541.00467-2021

Tables

TABLE 1

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.