Related Experiment Video
Updated: May 18, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
IgG4-related systemic disease presenting with lung entrapment and constrictive pericarditis
Hiroshi Sekiguchi1, Ryohei Horie1, James P Utz1
1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN.
Abstract:
We describe a 29-year-old woman who presented with chronic pleuropericarditis complicated by lung entrapment and constrictive pericarditis. Pleural biopsy performed during the decortication procedure revealed fibrinous pleuritis with lymphoplasmacytic inflammation including IgG4-positive plasma cells. The patient responded favorably to corticosteroid therapy with resolution of pleural effusion and constrictive physiology. To our knowledge, this is the first reported case of IgG4-related systemic disease manifesting as lung entrapment and constrictive pericarditis.
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