Rheumatology Commons^™

Challenges In Medicine: The Odyssey Of A Patient With Isolated Igg4-Related Eosinophilic Angiocentric Fibrosis Presenting As A Locally Destructive Sinonasal Mass, Snigdha Nutalapati, Richard O'Neal, William O'Connor, Brett T. Comer, Gerhard C. Hildebrandt

Otolaryngology--Head & Neck Surgery Faculty Publications

Eosinophilic angiocentric fibrosis (EAF) is an exceeding rare clinical entity and is considered a part of the spectrum of IgG4-related disease (IgG4RD). We hereby present such an unusual case of a 60-year-old female who presented to us with recurrent sinonasal mass, after a decade long haul of multiple clinical evaluations, biopsies, and debulking surgery without a definitive diagnosis. Over this period, the mass eroded through the ethmoid cells along with nasal septal destruction leading to saddle nose deformity, extended superiorly through the cribriform plates to right frontal lobe, and compressed the optic nerve leading to visual loss. Although initial biopsy …