Eosinophilic angiocentric fibrosis
Synonyms: IgG4-related eosinophilic angiocentric fibrosis
A rare otorhinolaryngologic disease characterized by an indolent submucosal mass of variable size and extent most commonly arising in the anterior nasal cavity involving the nasal septum and lateral nasal wall and potentially extending into the adjacent sinuses. Occurrence in the larynx and lower respiratory tract or the orbit is rare. Histological examination shows concentric angiocentric stromal fibrosis (onionskin fibrosis) and prominent eosinophils. Increased numbers of IgG4-positive plasma cells in the lesion may also be observed in addition to elevated serum IgG4. Patients typically present with long-standing obstructive symptoms.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
Newly diagnosed with
Eosinophilic angiocentric fibrosis?
Our RARE Concierge Services Guides are available to assist you by providing information, resources and connections as you navigate your rare disease journey.
Advocacy Organizations
Global Liver Institute
To improve the lives of individuals and families impacted by liver disease through promoting innovation, encouraging collaboration, and scaling optimal approaches to help eradicate liver diseases.
Clinical Trials
For a list of clinical trials in this disease area, please click here.