Lung and airway disorders in immunoglobulin G4- related disease

Arvelo Castro, Luis and Carmona Porquera, Eva M. and Jay H. Ryu, Jay H. (2015) Lung and airway disorders in immunoglobulin G4- related disease. Shortness of breath, 4 (3). 76 -87. ISSN 2281-6550

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gG4-related disease (IgG4-RD) is an immune-mediated condition, characterized by the formation of fibroinflammatory tumefactive lesions that can cause progressive tissue damage and subsequent organ failure. Although initially described as a disease of unknown etiology confined to the pancreas, IgG4-RD has been realized over time as an important entity that has underlies numerous pathologic conditions such as autoimmune pancreatitis, Riedel thyroiditis, Mikulicz syndrome, inflammatory pseudotumors and many others. IgG4-RD can affect almost any organ. However, the most commonly affected are the pancreas, hepatobiliary system, exocrine glands (salivary, lacri mal), retroperitoneum, kidneys and intrathoracic structures. Within the thorax, involvement of the lung parenchyma, airways, mediastinum, and pleura have been reported. The pulmonary involvement, therefore, is very diverse and can present as pulmonary nodule(s) or mass(es), alveolar or interstitial infiltrates, airflow obstruction, lymphadenopathy, mediastinal fibrosis, and pleural thickening and/or effusion. It is therefore important to recognize the diverse manifestations of this condition, as it can involve multiple organs and have various forms of expression within the same organ. As a consequence, IgG4-RD has presented a fascinating diagnostic challenge for many different specialties. In this review, we focus on the diagnosis and management of the intrathoracic involvement in this multisystem disease.

Item Type: Article
Uncontrolled Keywords: immunoglobulin G4, IgG4, fibroinflammatory, interstitial lung disease, lymphadenopathy.
Subjects: 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici)
Depositing User: Sig. Daniele Granieri
Date Deposited: 19 Mar 2020 15:05
Last Modified: 19 Mar 2020 15:05

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