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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.

Authors

John R. Hurst

Nisha Verma

David Lowe

Helen E. Baxendale

Stephen Jolles

Peter Kelleher

Hilary J. Longhurst

Smita Y. Patel

Elisabetta A. Renzoni

Clare R. Sander

Gerard R. Avery

Judith L. Babar

Matthew S. Buckland

Siobhan Burns

William Egner

Mark M. Gompels

Pavels Gordins

Jamanda A. Haddock

Simon P. Hart

Grant R. Hayman

Richard Herriot

Rachel K. Hoyles

Aarnoud P. Huissoon

Joseph Jacob

Andrew G. Nicholson

Doris M. Rassl

Ravishankar B. Sargur

Sinisa Savic

Suranjith L. Seneviratne

Michael Sheaff

Prashantha M. Vaitla

Gareth I. Walters

Joanna L. Whitehouse

Penny A. Wright

Alison M. Condliffe

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).

Journal Article Type Article
Publication Date Jul 1, 2017
Journal Journal of allergy and clinical immunology : in practice
Print ISSN 2213-2198
Electronic ISSN 2213-2201
Peer Reviewed Peer Reviewed
Volume 5
Issue 4
Pages 938-945
Institution Citation Hurst, J. R., Verma, N., Lowe, D., Baxendale, H. E., Jolles, S., Kelleher, P., …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. doi:10.1016/j.jaip.2017.01.021
DOI https://doi.org/10.1016/j.jaip.2017.01.021
Keywords Common variable immunodeficiency; Lung Disease; Interstitial; Complications
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.

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Copyright Statement
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).




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