IgG4-related systemic disease presenting with lung entrapment and constrictive pericarditis.

Sekiguchi, Hiroshi; Horie, Ryohei; Utz, James P; Ryu, Jay H · Chest · 2012

case_report · Level V

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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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