Thoracic involvement and imaging patterns in IgG4-related disease

Abstract

ObjectiveImmunoglobulin 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.MethodsThoracic 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.Results48 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.ConclusionThoracic involvement of IgG4-RD is heterogeneous and likely underestimated. The main thoracic CT scan patterns are peribronchovascular thickening and thoracic lymph nodes.

IgG4 related lung disease mimicking multiple pleural disseminated lung cancer

“…Because the patient was asymptomatic and the disease was stable, no specific treatment was indicated. We plan to monitor the patient and conduct periodic physical examinations, laboratory testing, and surveillance CT. As IgG4‐RLD can present with various types of radiological findings, 1 IgG4‐RLD is often misdiagnosed as lung cancer. IgG4‐RLD generally manifests as mild clinical symptoms, despite the degree of lesions and their spread, determined in the imaging analysis, and excessive cellular infiltration, observed during pathological examinations 2 .…”

Large lung mass lesion with spontaneous regression in a patient with IgG4‐related lung disease

“…Despite solid lung nodules being reported in 33% of patients with IgG4‐RLD, the frequency of a mass lesion occurrence is not accurately known. 2 Respirologists should be some IgG4‐RLD may not need steroid therapy.…”

Pulmonary IgG4‐related disease with favourable response to rituximab: A case report

“…Observational studies have shown that radiological presentation of pulmonary involvement in IgG4‐RD is polymorphic and often non‐specific. 2 …”

“…Pulmonary involvement is relatively less frequent but is probably underestimated. Observational studies have shown that radiological presentation of pulmonary involvement in IgG4‐RD is polymorphic and often non‐specific 2 …”

“…Observational studies have shown that radiological presentation of pulmonary involvement in IgG4-RD is polymorphic and often non-specific. 2 The disease is usually treated by steroids, with a remarkable efficacy but relapses are frequent. Second line treatments as monoclonal anti-CD20 antibodies are usually proposed in patients with relapses as steroid sparing agents.…”