Immunoglobulin G4-related sclerosing disease of the paranasal sinus

Robert H. Lindau, Yungpo Bernard Su, Roger Kobayashi, Russell B. Smith

Research output: Contribution to journalArticlepeer-review

24 Scopus citations


Background: Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. We present a case of a 69-year-old man with maxillary sinus IgG4 sclerosing disease, with orbital invasion treated with rituximab and dexamethasone pulse therapy. Surgery was used as well to debulk the disease and to obtain tissue for diagnosis. Methods: A PubMed search using the key phrase "IgG4-related Sclerosing Disease" was performed. There were 304 different articles regarding the disease for a multitude of different organ sites. Of the 304 articles, there were 3 articles that reported this disease in the paranasal sinuses. Conclusions: IgG4-related sclerosing disease is a rare entity in the head and neck. There are documented reports of steroid therapy for this disease, but the patient presented here demonstrated clinical progression of disease with steroids alone. The use of combination therapy of surgery, dexamethasone, and rituximab provided clinical improvement and stable disease determined by radiographic means. © 2012 Wiley Periodicals, Inc. Head Neck 35: E321-E324, 2013

Original languageEnglish (US)
Pages (from-to)E321-E324
JournalHead and Neck
Issue number10
StatePublished - Oct 2013


  • IgG4 plasma cells
  • IgG4 sclerosing disease
  • IgG4 sclerosing disease treatment
  • paranasal sinus
  • rituximab

ASJC Scopus subject areas

  • Otorhinolaryngology


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