Special Article
British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders

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A proportion of people living with common variable immunodeficiency disorders develop granulomatous-lymphocytic interstitial lung disease (GLILD). We aimed to develop a consensus statement on the definition, diagnosis, and management of GLILD. All UK specialist centers were contacted and relevant physicians were invited to take part in a 3-round online Delphi process. Responses were graded as Strongly Agree, Tend to Agree, Neither Agree nor Disagree, Tend to Disagree, and Strongly Disagree, scored +1, +0.5, 0, −0.5, and −1, respectively. Agreement was defined as greater than or equal to 80% consensus. Scores are reported as mean ± SD. There was 100% agreement (score, 0.92 ± 0.19) for the following definition: “GLILD is a distinct clinico-radio-pathological ILD occurring in patients with [common variable immunodeficiency disorders], associated with a lymphocytic infiltrate and/or granuloma in the lung, and in whom other conditions have been considered and where possible excluded.” There was consensus that the workup of suspected GLILD requires chest computed tomography (CT) (0.98 ± 0.01), lung function tests (eg, gas transfer, 0.94 ± 0.17), bronchoscopy to exclude infection (0.63 ± 0.50), and lung biopsy (0.58 ± 0.40). There was no consensus on whether expectant management following optimization of immunoglobulin therapy was acceptable: 67% agreed, 25% disagreed, score 0.38 ± 0.59; 90% agreed that when treatment was required, first-line treatment should be with corticosteroids alone (score, 0.55 ± 0.51).

Key words

Common variable immunodeficiency
Lung Disease
Interstitial
Complications

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The British Lung Foundation funded this work (grant reference no. PPRG15-7).

Conflicts of interest: J. R. Hurst has received research support from the British Lung Foundation; has received consultancy and lecture fees from AstraZeneca, Boehringer, Chiesi, Grifols, GlaxoSmithKline, Pfizer, Novartis, and Takeda; and has received travel support from AstraZeneca and Boehringer. D. Lowe has received consultancy fees and travel support from Biotest and has received research support from University College London Biomedical Research Centre. S. Jolles reports grants, personal fees, nonfinancial support, and other from CSL Behring and Baxalta; grants, personal fees, and nonfinancial support from Shire and Biotest; grants from Binding Site; grants and personal fees from Swedish Orphan Biovitrum; personal fees and nonfinancial support from Grifols, Octapharma, and UCB Pharma; personal fees from LFB Group; and nonfinancial support from Bio Products Laboratory Ltd (BPL), outside the submitted work. P. Kelleher has received research support from Imperial College Healthcare National Health Service (NHS) Trust, Cheslea & Westminster NHS Foundation Trust, and Royal Brompton & Harefield NHS Trust and has received research support from Engineering and Physical Sciences Research Council, St Stephen AIDS Trust, lmperial College Healthcare NHS Trust BRC, Chelsea & Westminster Health Charity, and Westminster Medical School Research Trust. H. J. Longhurst has received consultancy fees from CSL Behring, Biotest, and Shire/Baxalta; has received research support from CSL Behring, Shire/Baxalta, and Grifols; has received lecture fees from CSL Behring and Shire/Baxalta; has received payment for the development of educational presentations from CSL Behring; and has received travel support from CSL Behring, Shire/Baxalta, Octapharma, and Grifols. E. A. Renzoni has received lecture fees from Roche, Boehringer, and Takeda. M. S. Buckland has received consultancy fees from Octopharma. S. Burns has received research support from Higher Education Funding Council for England, National Institute for Health Research, University College London Hospitals Inflammation Immunity and Immunotherapeutics, and Great Ormond Street Hospital biomedical research centres; has received consultancy fees from CSL Behring; is employed by the University College of London; and has received travel support from Immunodeficiency Canada/International Association of Allergy and Clinical Immunology, CSL Behring, and Baxalta. M. M. Gompels has received consultancy fees from Biocryst for participation on an advisory board; is employed by NHS and private practice; has provided expert testimony for medicolegal work; has received research support from North Bristol NHS Trust; has received lecture fees and payment for developing educational presentations from Shire Pharmaceuticals; and has received travel support from CSL Behring and Novartis. P. Gordins has received consultancy fees and travel support from Biotest and has received travel support from BPL. A. P. Huissoon has received lecture fees from Immunodeficiency Forum and has received travel support from BPL, CSL Behring, and Biotest. A. G. Nicholson has received consultancy fees from Boehringer Ingelheim and Med Quantitative Image Analysis; is employed by Sanofi; and has received lecture fees from Roche and Intermune. D. M. Rassl has received consultancy fees from PDL Biopharma and has received research support from Cancer Research UK and Qiagen/Innovate UK. M. Sheaff has provided expert opinion in medicolegal cases. J. L. Whitehouse has received lecture fees from Chiesi and was on the advisory board for PTC Therapeutics. A. M. Condliffe has received research support from GlaxoSmithKline. The rest of the authors declare that they have no relevant conflicts of interest.