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Updated: Dec 23, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Nasal eosinophilic angiocentric fibrosis with IgG4-positive plasma cell infiltration
B Saenz-Ibarra1, L A Ceceñas-Falcon, J A Cardenas-De la Garza
1University Hospital Dr. Jose Eleuterio Gonzalez UANL, Anatomical Pathology and Cytopathology Department, Francisco I. Madero y Gonzalitos S/N, 64460 Monterrey, Nuevo Leon, Mexico. bsipatologia@gmail.com.
Introduction:
Eosinophilic angiocentric fibrosis (EAF) is a rare lesion that predominantly affects the upper respiratory tract. Its etiology is unknown and it has been recently associated with the IgG4- related disease (IgG4-RD) spectrum. To the author's knowledge, this is the sixth case report of the relationship between EAF and IgG4-RD.
Case Report:
We report the case of a 37-year-old woman with nasal deformity and facial pain. The lesion was surgically excised. Histological examination revealed features of EAF with an IgG4/IgG plasma cell ratio ≷73% and 31 IgG4 stained cells per high power field. No clinical or radiological recurrence was detected during follow-up. Serum IgG4 quantification one year after surgery was within normal limits.
Discussion:
The relationship between both entities may have therapeutic impact because IgG4-RD of the head and neck has a high remission rate with corticosteroids and immunosuppressive therapy. Additional reports of this infrequent disease are necessary to elucidate appropriate treatment and prognosis.
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