Chest
Volume 142, Issue 3, September 2012, Pages 781-783
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Selected Reports
IgG4-Related Systemic Disease Presenting With Lung Entrapment and Constrictive Pericarditis

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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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Case Presentation

A 29-year-old woman was in good general health until she developed intermittent pleuritic chest pain accompanied by mild exertional dyspnea 5 years before presentation to our clinic. Her symptoms remained mild until 1 year before, when she became increasingly dyspneic. A CT scan of the chest demonstrated bilateral pleural thickening and effusion, greater on the right. Laboratory evaluation results for infectious and rheumatologic diseases were negative except for a mildly elevated erythrocyte

Discussion

Although ISD was initially recognized as autoimmune pancreatitis, it has become apparent in recent years that this disorder can involve virtually any organ. Characteristic histopathologic features of ISD include the presence of abundant IgG4-positive lymphoplasmacytic infiltration, fibrosis, and obliterative phlebitis or arteritis.1, 2 However, obliterative phlebitis or arteritis may not be observed in the intrathoracic manifestations of this disease.2 The histopathologic findings and the

Conclusion

ISD can present with chronic pleuropericarditis, culminating in entrapped lung and constrictive pericarditis. Increased awareness of ISD and measurement of serum IgG4 levels may facilitate diagnosis and prevent morbidity associated with this disease as well as unnecessary diagnostic procedures. ISD should be considered in cases of unexplained pleuritis and pericarditis to avoid diagnostic delay and associated morbidity. Further studies are warranted to investigate the frequency of ISD

Acknowledgments

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

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