PT - JOURNAL ARTICLE AU - Muller, Romain AU - Habert, Paul AU - Ebbo, Mikael AU - Graveleau, Julie AU - Groh, Mathieu AU - Launay, David AU - Audia, Sylvain AU - Pugnet, Gregory AU - Cohen, Fleur AU - Perlat, Antoinette AU - Benyamine, Audrey AU - Bienvenu, Boris AU - Gaigne, Lea AU - Chanez, Pascal AU - Gaubert, Jean Yves AU - Schleinitz, Nicolas TI - Thoracic involvement and imaging patterns in IgG4-related disease AID - 10.1183/16000617.0078-2021 DP - 2021 Dec 31 TA - European Respiratory Review PG - 210078 VI - 30 IP - 162 4099 - https://publications.ersnet.org//content/30/162/210078.short 4100 - https://publications.ersnet.org//content/30/162/210078.full SO - EUROPEAN RESPIRATORY REVIEW2021 Dec 31; 30 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