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Updated: Aug 23, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary and coronary arterial abnormalities in patients with IgG4-related disease
Rory L Cochran1, Holly R Brideau1, Markus Y Wu1
1Department of Radiology, Massachusetts General Hospital, 55 Fruit St, Boston, MA, USA.
Abstract:
IgG4-related disease (IgG4-RD) is an immune-mediated multiorgan fibroinflammatory disorder with variable clinical presentations. IgG4-RD cardiovascular involvement is considered rare, with pulmonary arterial involvement reported in a small subset of cases. Known pulmonary artery manifestations include pulmonary arteritis, pulmonary artery stenoses and central pulmonary artery aneurysms. Here we report 2 different patients with multifocal dilatation of the segmental and subsegmental pulmonary arteries with differing degrees of severity. Both patients also had coronary arterial abnormalities.
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