417. Complete remission of Eosinophilic Angiocentric Fibrosis with Rituximab without glucocorticoids
Creators: name: Francesca Regola1, 2
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case:</strong> A 48-year-old Caucasian woman presented with an eight-year history of progressive severe nasal obstruction and occasional rhinorrhoea, left greater than right. Computerized tomography (CT) revealed a large hypodense obstructive mass in the anterior area of the left nasal cavity, associated with an erosion of the nasal septum. The patient underwent a resection of the neoformation and a septoplasty. Histological examination of the resected fragments of septal tissue revealed dense lamellar perivascular fibrosis with focal storiform fibrosis. Tissue infiltration by CD3+ lymphocytes, histiocytes, polyclonal plasmacells and, most of all, numerous eosinophils, was found, especially in the perivascular area. Immunohistochemistry staining showed rare IgG4 positive plasma cells, with a IgG4/IgG ratio lower than 1/40. The fibrotic tissue invaded the underline cartilaginous tissue and septal bone area. No evidence of granulomatous inflammation, necrosis, giant cells, or malignant change were found. After six months the patient reported nasal obstruction recurrence. A magnetic resonance imaging (MRI) confirmed the suspected relapse, showing a new 17x9 mm mass arising from the anterior aspect of the nasal septum. The patient underwent a second resection of the neoformation, and the histological examination was consistent with the first one. <strong>Diagnostic Testing: </strong>After the disease relapse, the patient was referred to our Vasculitic Clinic. Serum chemistries, erythrocyte sedimentation rate, C-reactive protein levels, white blood cell and eosinophil count, serum levels of IgG, IgG4, IgA, IgM, IgE, C3 and C4 were found normal. ANA, ENA, anti-dsDNA, ANCA, IGRA, were negative, as also skin prick allergy test. <strong>Differential &amp; Final Diagnosis:</strong> Based on laboratory tests and histological examination, infectious diseases, ANCA-associated vasculitis, sarcoidosis, and neoplastic conditions were excluded. Radiological and histological examination were consistent with a diagnosis of Eosinophilic angiocentric fibrosis (EAF). EAF is a rare entity, characterized by expansive lesions of sinonasal and upper respiratory tract. Its aetiology is unknown but a connection with IgG4- related diseases has been suggested (1). <strong>Discussion of Management</strong>: In general, the prognosis of sinonasal tract EAF seems to be indolent and progressive, but in some cases EAF could extend into the adjacent cartilaginous tissue and cause bone destruction, like in our patient. Until now, only isolated case reports on EAF have been published. Multiple surgical resections have been chosen as first-line therapeutic approach in most of these cases, while immunosuppressive therapy and glucocorticoids (GC) seemed to be associated with only limited success (2). Our patient refused to be treated with high dose GC, frightened by possible GC-related adverse effects and by the limited evidence of their efficacy in EAF. Therefore, Rituximab monotherapy was proposed. The patient was treated with Rituximab (RTX) 1,000 mg on days 1 and 15. Three months after the first RTX infusion she repeated an MRI scan and an ENT evaluation, demonstrating no radiological or endoscopic sign of relapse. Based on the efficacy of the induction treatment, RTX 1,000 mg every 6 months was chosen as maintenance therapy. Subsequent MRI scans and ENT evaluations after 10, 18 and 24 months after the first RTX infusion showed persistent disease remission. <strong>Conclusions:</strong> EAF is considered a rare, slowly progressive, and benign disease but in some patients can be locally aggressive and relapsing. A systemic treatment might be necessary in these patients and RTX proved to be effective in our patient, even in a monotherapy regiment. <strong>Disclosures:</strong> None