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.
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).
authors.affiliation: UCL Respiratory, University College London, London, United Kingdom. Electronic address: j.hurst@ucl.ac.uk.
authors.forename: John R
authors.initials: JR
authors.lastname: Hurst
authors.affiliation: Institute of Immunity and Transplantation, Royal Free Hospital, London, United Kingdom.
authors.forename: Nisha
authors.initials: N
authors.lastname: Verma
authors.affiliation: Institute of Immunity and Transplantation, Royal Free Hospital, London, United Kingdom.
authors.forename: David
authors.initials: D
authors.lastname: Lowe
authors.affiliation: Cambridge Centre for Lung Defense, Papworth Hospital, Cambridge, United Kingdom.
authors.forename: Helen E
authors.initials: HE
authors.lastname: Baxendale
authors.affiliation: Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, United Kingdom.
authors.forename: Stephen
authors.initials: S
authors.lastname: Jolles
authors.affiliation: Centre for Immunology and Vaccinology, Imperial College and Department of Respiratory Medicine, Royal Brompton & Harefield Hospitals NHS Foundation Trust, London, United Kingdom.
authors.forename: Peter
authors.initials: P
authors.lastname: Kelleher
authors.affiliation: Department of Immunology, Barts Health NHS Trust, London, United Kingdom.
authors.forename: Hilary J
authors.initials: HJ
authors.lastname: Longhurst
authors.affiliation: Oxford NIHR Biomedical Research Centre, Oxford, United Kingdom.
authors.forename: Smita Y
authors.initials: SY
authors.lastname: Patel
authors.affiliation: Interstitial Lung Disease Unit, Royal Brompton & Harefield Hospitals NHS Foundation Trust, London, United Kingdom.
authors.forename: Elisabetta A
authors.initials: EA
authors.lastname: Renzoni
authors.affiliation: Respiratory Medicine, Cambridge University NHS Foundation Trust, Cambridge, United Kingdom.
authors.forename: Clare R
authors.initials: CR
authors.lastname: Sander
authors.affiliation: Department of Radiology, Hull and East Yorkshire NHS Trust, Hull, United Kingdom.
authors.forename: Gerard R
authors.initials: GR
authors.lastname: Avery
authors.affiliation: Department of Radiology, Cambridge University NHS Foundation Trust, Cambridge, United Kingdom.
authors.forename: Judith L
authors.initials: JL
authors.lastname: Babar
authors.affiliation: Institute of Immunity and Transplantation, Royal Free Hospital, London, United Kingdom.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Buckland
authors.affiliation: Institute of Immunity and Transplantation, Royal Free Hospital, London, United Kingdom.
authors.forename: Siobhan
authors.initials: S
authors.lastname: Burns
authors.affiliation: Clinical Immunology and Allergy Unit, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom.
authors.forename: William
authors.initials: W
authors.lastname: Egner
authors.affiliation: Department of Immunology, North Bristol NHS Trust, Bristol, United Kingdom.
authors.forename: Mark M
authors.initials: MM
authors.lastname: Gompels
authors.affiliation: East Yorkshire Regional Adult Immunology and Allergy Unit, Castle Hill Hospital, Hull, United Kingdom.
authors.forename: Pavels
authors.initials: P
authors.lastname: Gordins
authors.affiliation: Department of Radiology, Royal Free London NHS Foundation Trust, London, United Kingdom.
authors.forename: Jamanda A
authors.initials: JA
authors.lastname: Haddock
authors.affiliation: Hull York Medical School, University of Hull, Castle Hill Hospital, Hull, United Kingdom.
authors.forename: Simon P
authors.initials: SP
authors.lastname: Hart
authors.affiliation: Department of Immunology, Epsom & St Helier University Hospitals NHS Trust, Epsom, United Kingdom.
authors.forename: Grant R
authors.initials: GR
authors.lastname: Hayman
authors.affiliation: Immunology Department, Aberdeen Royal Infirmary, Aberdeen, United Kingdom.
authors.forename: Richard
authors.initials: R
authors.lastname: Herriot
authors.affiliation: Oxford Centre for Respiratory Medicine, Churchill Hospital, Oxford, United Kingdom.
authors.forename: Rachel K
authors.initials: RK
authors.lastname: Hoyles
authors.affiliation: West Midlands Immunodeficiency Centre, Birmingham Heartlands Hospital, Birmingham, United Kingdom.
authors.forename: Aarnoud P
authors.initials: AP
authors.lastname: Huissoon
authors.affiliation: Department of Radiology, Royal Free London NHS Foundation Trust, London, United Kingdom.
authors.forename: Joseph
authors.initials: J
authors.lastname: Jacob
authors.affiliation: Department of Histopathology, Royal Brompton & Harefield Hospitals NHS Foundation Trust, and National Heart and Lung Institute, Imperial College, London, United Kingdom.
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Nicholson
authors.affiliation: Department of Pathology, Papworth Hospital, Cambridge, United Kingdom.
authors.forename: Doris M
authors.initials: DM
authors.lastname: Rassl
authors.affiliation: Clinical Immunology and Allergy Unit, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom.
authors.forename: Ravishankar B
authors.initials: RB
authors.lastname: Sargur
authors.affiliation: Department of Clinical Immunology and Allergy, St James's University Hospital, Leeds, United Kingdom.
authors.forename: Sinisa
authors.initials: S
authors.lastname: Savic
authors.affiliation: Institute of Immunity and Transplantation, Royal Free Hospital, London, United Kingdom.
authors.forename: Suranjith L
authors.initials: SL
authors.lastname: Seneviratne
authors.affiliation: Department of Cellular Pathology, Barts Health NHS Trust, London, United Kingdom.
authors.forename: Michael
authors.initials: M
authors.lastname: Sheaff
authors.affiliation: Department of Immunology, Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom.
authors.forename: Prashantha M
authors.initials: PM
authors.lastname: Vaitla
authors.affiliation: Regional Occupational Lung Disease Service, Birmingham Chest Clinic, Birmingham, United Kingdom.
authors.forename: Gareth I
authors.initials: GI
authors.lastname: Walters
authors.affiliation: Respiratory Medicine, Heart of England NHS Foundation Trust, Birmingham, United Kingdom.
authors.forename: Joanna L
authors.initials: JL
authors.lastname: Whitehouse
authors.affiliation: Histopathology Department, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom.
authors.forename: Penny A
authors.initials: PA
authors.lastname: Wright
authors.affiliation: Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom.
authors.forename: Alison M
authors.initials: AM
authors.lastname: Condliffe
mesh_terms: D002608:Charities; D017074:Common Variable Immunodeficiency / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D032921:Consensus; D006099:Granuloma / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D012955:Societies, Medical; D006113:United Kingdom
keywords: Common variable immunodeficiency; Complications; Interstitial; Lung Disease