• 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, and Alison M Condliffe.
• UCL Respiratory, University College London, London, United Kingdom. Electronic address: j.hurst@ucl.ac.uk.
• J Allergy Clin Immunol Pract. 2017 Jul 1; 5 (4): 938-945.

AbstractA 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).Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

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