Premium
Immunoglobulin G4–related sclerosing disease of the paranasal sinus
Author(s) -
Lindau Robert H.,
Su Yungpo Bernard,
Kobayashi Roger,
Smith Russell B.
Publication year - 2013
Publication title -
head and neck
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.012
H-Index - 127
eISSN - 1097-0347
pISSN - 1043-3074
DOI - 10.1002/hed.23175
Subject(s) - medicine , rituximab , disease , igg4 related disease , paranasal sinuses , pathology , sinusitis , sinus (botany) , lymphoma , surgery , botany , biology , genus
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