Nasal manifestations of IgG4-related disease: A report of two cases

Auris Nasus Larynx. 2015 Dec;42(6):483-7. doi: 10.1016/j.anl.2015.05.004. Epub 2015 Jun 6.

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized clinical disease entity characterized by elevated serum IgG4, tumefaction, tissue infiltration of IgG4-positive plasma cells and fibrosis. IgG4-RD may occur, either synchronously or metachronously, in a variety of organs throughout the body. We describe herein two representative cases of the nasal manifestations of IgG4-RD, characterized by diffuse, crusty, erosive lesions on nasal mucosa. Oral steroid administration was effective in treating these nasal manifestations. We report a decrease in IgG4 positive plasma cell infiltrates in nasal mucosa biopsy specimens after steroid therapy, demonstrating that infiltration of IgG4-positive cells is reversible.

Keywords: IgG4; IgG4-related disease; Immunoglobulin; Nasal manifestations; Rhinitis; Rhinosinusitis.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology
  • Humans
  • Immunoglobulin G / immunology*
  • Male
  • Middle Aged
  • Nasal Mucosa / immunology*
  • Nasal Mucosa / pathology
  • Rhinitis / drug therapy
  • Rhinitis / immunology*
  • Rhinitis / pathology
  • Sinusitis / drug therapy
  • Sinusitis / immunology*
  • Sinusitis / pathology

Substances

  • Adrenal Cortex Hormones
  • Immunoglobulin G