Immunoglobulin G4 –related Disease

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 l ung 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.

Source: Clinics in Chest Medicine - August 1, 2019 Category: Respiratory Medicine Authors: Zachary S. Wallace, Cory Perugino, Mark Matza, Vikram Deshpande, Amita Sharma, John H. Stone Source Type: research