IgG4-Related Lung Disease

  • Jay H. Ryu
  • Hiroshi Sekiguchi
  • Eunhee S. Yi


Pulmonary involvement in IgG4-related disease can manifest in any intrathoracic compartment including the lung parenchyma, airways, lymph nodes, mediastinum, and pleura. Although most patients with IgG4-related lung disease have extrapulmonary manifestations, some patients present with isolated pulmonary disease and may be difficult to diagnose. Approximately one-half of those with IgG4-related lung disease have respiratory symptoms at presentation. The most common form of intrathoracic involvement is mediastinal and/or hilar lymphadenopathy. Parenchymal manifestations of IgG4-related lung disease include single or multiple rounded opacities and interstitial lung disease. Airway disease can cause airway narrowing and asthma-like symptoms. Aside from mediastinal lymphadenopathy, IgG4-related fibrosing mediastinitis has also been reported. Pleural manifestations include pleural masses and pleural effusions. These patterns of intrathoracic disease are best characterized by CT (including high-resolution images of the lung parenchyma). Diagnostic confirmation of IgG4-related lung disease may require bronchoscopic or surgical biopsy, partly depending on the site of involvement. Histopathologic findings associated with IgG4-related lung disease are similar to those seen in extrapulmonary organs, but characteristic storiform fibrosis is not as apparent in lung biopsies. IgG4-related lung disease generally responds well to corticosteroid therapy.


Idiopathic Pulmonary Fibrosis Interstitial Lung Disease Serum IgG4 Level Idiopathic Interstitial Pneumonia Hilar Lymphadenopathy 
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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Division of Pulmonary and Critical Care MedicineMayo Clinic College of MedicineRochesterUSA
  2. 2.Division of Pulmonary and Critical Care MedicineMayo Clinic RochesterRochesterUSA
  3. 3.Department of Laboratory Medicine and PathologyMayo ClinicRochesterUSA

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