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J Allergy Clin Immunol Pract. 2017 Jul - Aug;5(4):938-945. doi: 10.1016/j.jaip.2017.01.021. Epub 2017 Mar 25.

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.

Author information

1
UCL Respiratory, University College London, London, United Kingdom. Electronic address: j.hurst@ucl.ac.uk.
2
Institute of Immunity and Transplantation, Royal Free Hospital, London, United Kingdom.
3
Cambridge Centre for Lung Defense, Papworth Hospital, Cambridge, United Kingdom.
4
Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, United Kingdom.
5
Centre for Immunology and Vaccinology, Imperial College and Department of Respiratory Medicine, Royal Brompton & Harefield Hospitals NHS Foundation Trust, London, United Kingdom.
6
Department of Immunology, Barts Health NHS Trust, London, United Kingdom.
7
Oxford NIHR Biomedical Research Centre, Oxford, United Kingdom.
8
Interstitial Lung Disease Unit, Royal Brompton & Harefield Hospitals NHS Foundation Trust, London, United Kingdom.
9
Respiratory Medicine, Cambridge University NHS Foundation Trust, Cambridge, United Kingdom.
10
Department of Radiology, Hull and East Yorkshire NHS Trust, Hull, United Kingdom.
11
Department of Radiology, Cambridge University NHS Foundation Trust, Cambridge, United Kingdom.
12
Clinical Immunology and Allergy Unit, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom.
13
Department of Immunology, North Bristol NHS Trust, Bristol, United Kingdom.
14
East Yorkshire Regional Adult Immunology and Allergy Unit, Castle Hill Hospital, Hull, United Kingdom.
15
Department of Radiology, Royal Free London NHS Foundation Trust, London, United Kingdom.
16
Hull York Medical School, University of Hull, Castle Hill Hospital, Hull, United Kingdom.
17
Department of Immunology, Epsom & St Helier University Hospitals NHS Trust, Epsom, United Kingdom.
18
Immunology Department, Aberdeen Royal Infirmary, Aberdeen, United Kingdom.
19
Oxford Centre for Respiratory Medicine, Churchill Hospital, Oxford, United Kingdom.
20
West Midlands Immunodeficiency Centre, Birmingham Heartlands Hospital, Birmingham, United Kingdom.
21
Department of Histopathology, Royal Brompton & Harefield Hospitals NHS Foundation Trust, and National Heart and Lung Institute, Imperial College, London, United Kingdom.
22
Department of Pathology, Papworth Hospital, Cambridge, United Kingdom.
23
Department of Clinical Immunology and Allergy, St James's University Hospital, Leeds, United Kingdom.
24
Department of Cellular Pathology, Barts Health NHS Trust, London, United Kingdom.
25
Department of Immunology, Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom.
26
Regional Occupational Lung Disease Service, Birmingham Chest Clinic, Birmingham, United Kingdom.
27
Respiratory Medicine, Heart of England NHS Foundation Trust, Birmingham, United Kingdom.
28
Histopathology Department, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom.
29
Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom.

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).

KEYWORDS:

Common variable immunodeficiency; Complications; Interstitial; Lung Disease

PMID:
28351785
DOI:
10.1016/j.jaip.2017.01.021
[Indexed for MEDLINE]
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