Challenges in Medicine: The Odyssey of a Patient with Isolated IgG4-Related Eosinophilic Angiocentric Fibrosis Presenting as a Locally Destructive Sinonasal Mass

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 was negative, repeat biopsy was performed owing to high clinical suspicion due to all the classic histopathological findings compatible with the diagnosis of eosinophilic angiocentric fibrosis IgG4-related disease (EAF-IgG4RD). Steroids are the recommended first-line therapy; however, our case was resistant to steroids needing rituximab to halt the disease progression. Our case interestingly also had T-cell clonality and isolated isocitrate dehydrogenase 2 enzyme mutation on next-generation sequencing, suggesting a possible role of novel molecular-targeted therapies in this rare disease. This case highlights the clinical challenges physicians face towards diagnosing and treating EAF-IgG4RD, emphasizing the need for high clinical suspicion and the possible role of targeted therapies for this rare disease.

Eosinophilic Angiocentric Fibrosis Invading the Nasal Septum: A Case Report and Review of Literature

Eosinophilic angiocentric fibrosis (EAF) is a rare and slowly progressive disease, which usually involves the sino-nasal structures and upper respiratory tract. It is a fibroinflammatory lesion with an unclear etiology. Recent literature suggests a relation to rheumatic or immunological disorders. Therefore, immunophenotypic workup is critical when suspected. We report a case of a 32-year-old man complaining of nasal obstruction lasting more than 2 years. Nasal endoscopy and computed tomography showed a deviated septum with bilateral soft tissue swelling. During the septoturbinoplasty, a submucosal mass with severe adhesion was observed beneath the septal flap. The mass was completely removed. Dense stromal fibrosis with eosinophil-rich inflammatory cell infiltration was found on histologic examination and the patient was diagnosed with EAF. In addition, we reviewed the pathologic diagnostic criteria, differential diagnosis, and management of EAF.

Eosinophilic Angiocentric Fibrosis: A Review and Update of Its Association With Immunoglobulin G4–Related Disease

Eosinophilic angiocentric fibrosis is a rare indolent lesion of the head and neck region that has characteristic histologic findings of onionskin fibrosis and prominent eosinophils. Its pathogenesis has been poorly understood and has been most commonly attributed to hypersensitivity or previous trauma. Recently, the lesion has been included in the spectrum of immunoglobulin G4 (IgG4)–related disease. However, few of the existing cases of eosinophilic angiocentric fibrosis have been evaluated for IgG4+ and IgG+ plasma cells. Therefore, we provide an update on the clinical and histologic features of eosinophilic angiocentric fibrosis to increase awareness of the entity and encourage its further characterization as an IgG4-related disease.