title: Clinical review of pulmonary manifestations of IgG4-related disease.
abstract: IgG4-related disease (IgG4-RD) is a recently recognized systemic disease characterized by tumefactive lesions in various organ systems. The list of organs that can be involved continues to expand, and recently computed tomography (CT) descriptions of the pulmonary lesions found in the disease have been described. The clinical symptoms are nonspecific and may include cough, dyspnea, chest pain, and fever. The appropriate clinical presentation along with elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and storiform fibrosis is consistent with the disease. Steroids are used to treat this disease in addition to immunosupressives such as cyclosporine or rituxumab for steroid refractory disease. The pulmonary manifestations and imaging features can often mimic malignancy, and as such knowledge of the diagnostic, clinicopathologic, and radiographic features of the disease is required in order to provide appropriate diagnostic workup and treatment.
authors.affiliation: Department of Pulmonary, Critical Care, and Sleep Medicine, Carilion Clinic/Virginia Tech Carilion School of Medicine, Roanoke, Virginia.
authors.forename: Sabrina N
authors.initials: SN
authors.lastname: Campbell
authors.forename: Edmundo
authors.initials: E
authors.lastname: Rubio
authors.forename: A Lukas
authors.initials: AL
authors.lastname: Loschner
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D005355:Fibrosis; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D010950:Plasma Cells / Q000473:pathology
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents
keywords: Ig; IgG4; IgG4-related disease; pulmonary IgG4