title: Immunoglobulin G4-Related Disease and the Lung.
abstract: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease with protean manifestations involving virtually any organ in the body. At initial clinical presentation, 1 or multiple organs may be involved. Initial descriptions focused on pancreatic disease. It has, however, become clear that IgG4-RD can cause an immune-mediated fibroinflammatory process, commonly manifesting as mass-like lesions, in various regions of the body including the thorax where any compartment can be involved. This pathologic process is characterized by infiltration of IgG4+ plasma cells and a propensity to fibrosis leading to organ dysfunction which can be prevented by early diagnosis and corticosteroid therapy.
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Gonda 18 South, 200 First Street Southwest, Rochester, MN 55905, USA. Electronic address: ryu.jay@mayo.edu.
authors: Jay H
authors: JH
authors: Ryu
authors: Division of Anatomic Pathology, Mayo Clinic, Hilton Building 11, 200 First Street Southwest, Rochester, MN 55905, USA.
authors: Eunhee S
authors: ES
authors: Yi
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D042241:Early Diagnosis; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D010950:Plasma Cells / Q000276:immunology*
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G
keywords: Fibroinflammatory; Immunoglobulin G4; Interstitial lung disease; Lymphadenopathy; Pleuritis; Pseudotumor