Immunoglobulin G4-related Disease

Zachary S Wallace; Cory Perugino; Mark Matza; Vikram Deshpande; Amita Sharma; John H Stone

doi:10.1016/j.ccm.2019.05.005

Immunoglobulin G4-related Disease

Clin Chest Med. 2019 Sep;40(3):583-597. doi: 10.1016/j.ccm.2019.05.005.

Authors

Zachary S Wallace¹, Cory Perugino², Mark Matza³, Vikram Deshpande⁴, Amita Sharma⁵, John H Stone²

Affiliations

¹ Clinical Epidemiology Program, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA; Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA. Electronic address: zswallace@mgh.harvard.edu.
² Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA.
³ Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
⁴ Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA.
⁵ Department of Radiology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA 02114, USA.

Abstract

Immunoglobulin G4 (IgG4)-Related Disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ and lead to organ dysfunction and irreversible damage. In addition to frequent involvement of the salivary glands, lacrimal glands, and/or pancreas, IgG4-RD often affects the chest. Thoracic manifestations include lung nodules and consolidations, pleural thickening, aortitis, and lymphadenopathy. The diagnosis is made after careful clinicopathologic correlation because there is no single diagnostic test with excellent sensitivity or specificity. Biopsy of pulmonary lesions can be useful for distinguishing IgG4-RD from common mimickers. Immunosuppressive regimens, such as glucocorticoids and/or glucocorticoid-sparing agents, form the cornerstone of treatment.

Keywords: Chest; IgG4-related disease; Pulmonary.

Publication types

Review

MeSH terms

Humans
Immunoglobulin G4-Related Disease*
Lung / pathology*

Abstract

Publication types

MeSH terms

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