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.
File(s)JACI paper 2017.pdf (278.86 KB)
Published version
Author(s)
Type
Journal Article
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).
Date Issued
2017-03-25
Date Acceptance
2017-01-24
Citation
Journal of Allergy and Clinical Immunology: In Practice, 2017, 5 (4), pp.938-945
ISSN
2213-2198
Publisher
Elsevier
Start Page
938
End Page
945
Journal / Book Title
Journal of Allergy and Clinical Immunology: In Practice
Volume
5
Issue
4
Copyright Statement
© 2017 The Authors. Published by
Elsevier Inc. on behalf of the American Academy of Allergy,
Asthma & Immunology. This is an open access article under the
CC BY-NC-ND license (http://creativecommons.org/licenses/bync-nd/4.0/).
Elsevier Inc. on behalf of the American Academy of Allergy,
Asthma & Immunology. This is an open access article under the
CC BY-NC-ND license (http://creativecommons.org/licenses/bync-nd/4.0/).
Identifier
PII: S2213-2198(17)30035-1
Subjects
Science & Technology
Life Sciences & Biomedicine
Allergy
Immunology
Common variable immunodeficiency
Lung Disease
Interstitial
Complications
IMMUNE DYSREGULATION
PULMONARY
HYPOGAMMAGLOBULINEMIA
ASSOCIATIONS
PNEUMONITIS
MUTATIONS
CELLS
CTLA4
Publication Status
Published