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

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 cell...

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Bibliographic Details
Published inChest Vol. 142; no. 3; p. 781
Main Authors Sekiguchi, Hiroshi, Horie, Ryohei, Utz, James P, Ryu, Jay H
Format Journal Article
LanguageEnglish
Published United States 01.09.2012
Subjects
Adrenal Cortex Hormones - therapeutic use
Adult
Biopsy
Female
Humans
Immunoglobulin G - metabolism
Lung - metabolism
Lung - pathology
Pericarditis - diagnosis
Pericarditis - drug therapy
Pericarditis - immunology
Pericarditis, Constrictive - diagnosis
Pericarditis, Constrictive - drug therapy
Pericarditis, Constrictive - immunology
Plasma Cells - metabolism
Plasma Cells - pathology
Pleurisy - diagnosis
Pleurisy - drug therapy
Pleurisy - immunology
Tomography, X-Ray Computed
Treatment Outcome
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Summary: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.
ISSN:1931-3543
DOI:10.1378/chest.11-2608