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

Chest. 2012 Sep;142(3):781-783. doi: 10.1378/chest.11-2608.

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.

Publication types

  • Case Reports

MeSH terms

  • 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

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulin G