RT Journal Article
SR Electronic
T1 Thoracic involvement and imaging patterns in IgG4-related disease
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 210078
DO 10.1183/16000617.0078-2021
VO 30
IS 162
A1 Romain Muller
A1 Paul Habert
A1 Mikael Ebbo
A1 Julie Graveleau
A1 Mathieu Groh
A1 David Launay
A1 Sylvain Audia
A1 Gregory Pugnet
A1 Fleur Cohen
A1 Antoinette Perlat
A1 Audrey Benyamine
A1 Boris Bienvenu
A1 Lea Gaigne
A1 Pascal Chanez
A1 Jean Yves Gaubert
A1 Nicolas Schleinitz
YR 2021
UL http://err.ersjournals.com/content/30/162/210078.abstract
AB Objective Immunoglobulin G4-related disease (IgG4-RD) is a rare orphan disease. Lung, pleura, pericardium, mediastinum, aorta and lymph node involvement has been reported with variable frequency and mostly in Asian studies. The objective of this study was to describe thoracic involvement assessed by high-resolution thoracic computed tomography (CT) in Caucasian patients with IgG4-RD.Methods Thoracic CT scans before treatment were retrospectively collected through the French case registry of IgG4-RD and a single tertiary referral centre. CT scans were reviewed by two experts in thoracic imagery blinded from clinical data.Results 48 IgG4-RD patients with thoracic involvement were analysed. All had American College of Rheumatology/European League Against Rheumatism classification scores ≥20 and comprehensive diagnostic criteria for IgG4-RD. CT scan findings showed heterogeneous lesions. Seven patterns were observed: peribronchovascular involvement (56%), lymph node enlargement (31%), nodular disease (25%), interstitial disease (25%), ground-glass opacities (10%), pleural disease (8%) and retromediastinal fibrosis (4%). In 37% of cases two or more patterns were associated. Asthma was significantly associated with peribronchovascular involvement (p=0.04). Among eight patients evaluated by CT scan before and after treatments, only two patients with interstitial disease displayed no improvement.Conclusion Thoracic involvement of IgG4-RD is heterogeneous and likely underestimated. The main thoracic CT scan patterns are peribronchovascular thickening and thoracic lymph nodes.Thoracic and lung involvements of IgG4-RD are heterogeneous and under-evaluated. Peribronchovascular involvement and lymph node enlargement are most frequent patterns. Interstitial disease pattern seems to be associated with a lower rate of treatment response. https://bit.ly/3B54HiR