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

Hurst, John R.; Verma, Nisha; Lowe, David; Baxendale, Helen E.; Jolles, Stephen; Kelleher, Peter; Longhurst, Hilary J.; Patel, Smita Y.; Renzoni, Elisabetta A.; Sander, Clare R.; Avery, Gerard R.; Babar, Judith L.; Buckland, Matthew S.; Burns, Siobhan; Egner, William; Gompels, Mark M.; Gordins, Pavels; Haddock, Jamanda A.; Hart, Simon P.; Hayman, Grant R.; Herriot, Richard; Hoyles, Rachel K.; Huissoon, Aarnoud P.; Jacob, Joseph; Nicholson, Andrew G.; Rassl, Doris M.; Sargur, Ravishankar B.; Savic, Sinisa; Seneviratne, Suranjith L.; Sheaff, Michael; Vaitla, Prashantha M.; Walters, Gareth I.; Whitehouse, Joanna L.; Wright, Penny A.; Condliffe, Alison M.

Abstract

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).

Citation

Hurst, J. R., Verma, N., Lowe, D., Baxendale, H. E., Jolles, S., Kelleher, P., Longhurst, H. J., Patel, S. Y., Renzoni, E. A., Sander, C. R., Avery, G. R., Babar, J. L., Buckland, M. S., Burns, S., Egner, W., Gompels, M. M., Gordins, P., Haddock, J. A., Hart, S. P., Hayman, G. R., …Condliffe, A. M. (2017). 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. The journal of allergy and clinical immunology. In practice, 5(4), 938-945. https://doi.org/10.1016/j.jaip.2017.01.021

• Journal Article Type: Article
• Acceptance Date: Jan 24, 2017
• Online Publication Date: Mar 25, 2017
• Publication Date: Jul 1, 2017
• Deposit Date: Jun 16, 2017
• Publicly Available Date: Jun 16, 2017
• Journal: Journal of allergy and clinical immunology : in practice
• Print ISSN: 2213-2198
• Peer Reviewed: Peer Reviewed
• Volume: 5
• Issue: 4
• Pages: 938-945
• DOI: https://doi.org/10.1016/j.jaip.2017.01.021
• Keywords: Common variable immunodeficiency; Lung Disease; Interstitial; Complications
• Public URL: https://hull-repository.worktribe.com/output/452512
• Publisher URL: https://www.sciencedirect.com/science/article/pii/S2213219817300351
• Additional Information: This article is maintained by: Elsevier; Article Title: 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; Journal Title: The Journal of Allergy and Clinical Immunology: In Practice; CrossRef DOI link to publisher maintained version: http://dx.doi.org/10.1016/j.jaip.2017.01.021; Content Type: article; Copyright: 38; Immunology.
• Contract Date: Jun 16, 2017