Thoracic involvement and imaging patterns in IgG4-related disease

Autor: Romain Muller, Paul Habert, Mikael Ebbo, Julie Graveleau, Mathieu Groh, David Launay, Sylvain Audia, Gregory Pugnet, Fleur Cohen, Antoinette Perlat, Audrey Benyamine, Boris Bienvenu, Lea Gaigne, Pascal Chanez, Jean Yves Gaubert, Nicolas Schleinitz
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: European Respiratory Review, Vol 30, Iss 162 (2021)
Druh dokumentu: article
ISSN: 0905-9180
1600-0617
16000617
DOI: 10.1183/16000617.0078-2021
Popis: 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.
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