Recommended Citation
Nallani P, Guo W, Mayerhoff RM, and Meysami A. An Unusual Presentation of Immunoglobulin G4-Related Disease (IgG4-RD) Causing Subglottic Stenosis. Cureus 2022; 14(6):e26250.
Document Type
Article
Publication Date
6-1-2022
Publication Title
Cureus
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that is known to involve multiple organs and was first described as an entity in 2003. It is characterized by lesions with a dense lymphoplasmacytic infiltrate, IgG4-positive plasma cells, storiform fibrosis, and frequently elevated serum IgG4 levels. Organs that are commonly involved include the pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, retroperitoneum, lungs, pleura, thyroid, aorta, and lymph nodes. Rarer manifestations of IgG4-RD include central nervous system (CNS) involvement, prostatitis, mastitis, midline destructive disease, and nasopharyngeal disease. In this report, we discuss an atypical case of a young woman with laryngeal subglottic involvement leading to stenosis and airway obstruction, which was ultimately successfully managed with systemic immunosuppression.
PubMed ID
35911268
Volume
14
Issue
6
First Page
26250
Last Page
26250
