Cases Of Eosinophilic Angiocentric Fibrosis Research Articles

Eosinophilic Angiocentric Fibrosis of the Orbit: A Clinicopathologic Review of 6 Novel Cases With Review of the Literature

To describe 6 cases and review the current state of knowledge of eosinophilic angiocentric fibrosis (EAF) involving the orbit. Retrospective clinicopathologic case series and review of the current literature METHODS: Clinical records and histopathologic data of orbit-involving EAF were gathered between 2004 and 2022 from a single academic institution. The patients' presenting clinical symptoms and signs, laboratory data, radiographic studies, and management documentation were collected. Retrospective review identified 6 novel cases, totaling 31 cases of EAF involving the orbit described as of this writing. Fourteen patients were male, and the average age of presentation was 49.8 years (range 25-78 years). Eighteen patients had concurrent sinonasal involvement, whereas 13 had primary orbital involvement. The median duration of symptoms prior to evaluation was 24 months, with nasal symptoms, proptosis, periorbital swelling, and pain being the most common presenting symptoms. The majority of patients underwent surgical debulking, as well as treatment with glucocorticoids and steroid-sparing agents, such as rituximab, with varied results. EAF involving the orbit is uncommon. The histopathologic findings include a perivascular, eosinophil-rich infiltrate and a pauci-inflammatory storiform type of fibrosis concentrated around small vessels. Orbital involvement usually results from local extension from adjacent sinuses, but primary orbital involvement has been described. Surgical debulking and immunosuppressive agents such as rituximab have been shown to stabilize disease.

Eosinophilic Angiocentric Fibrosis in Bilateral Upper Eyelid Conjunctivas: A First Case Report

Patient: Female, 55-year-oldFinal Diagnosis: Eosinophilic angiocentric fibrosisSymptoms: Lid swellingMedication: Topical eye dropsClinical Procedure: Resection of conjunctivasSpecialty: OphthalmologyObjective:Rare diseaseBackground:Eosinophilic angiocentric fibrosis (EAF) is an extremely rare disease with characteristic histopathological findings of fibrotic onion-skin appearance and eosinophils. The lesion primarily affects the nasal cavity, paranasal sinus, and orbit. Although there have been approximately 78 cases of EAF reported in the literature to date, no cases of EAF in the eyelid conjunctiva have ever been reported.Case Report:Herein, we describe the case of a 55-year-old Japanese woman with a history of eosinophilic sinusitis and EAF in bilateral upper eyelid conjunctivas who underwent surgical resection of the affected tissue. Histopathological examination revealed collagen bundles winding around the vessels in an onion-skin pattern, and the presence of eosinophils, lymphocytes, and plasma cells.Conclusions:We describe the first reported case of EAF in bilateral upper eyelid conjunctivas. It can be successfully treated by surgical resection, and with no recurrence within 6 months postoperatively.

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.

A surgical case of eosinophilic angiocentric fibrosis of the lung.

Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory disease that develops from the respiratory organs and affects them. Almost all reports about EAF describe lesions affecting the upper respiratory tract. We present the first case of EAF of the lung treated by surgical excision. A 69-year-old female consulted our hospital following the detection of an abnormal chest shadow with chronic cough. Chest computed tomography showed a pulmonary growing mass in the right hilar area, which corresponded to an enhanced accumulation on positron emission tomography. We doubted a pulmonary malignant tumor and performed a right upper lobectomy. Pathological and other clinical presentations revealed EAF of the lung without coexisting systemic diseases. The patient had an uncomplicated postoperative course, and the presenting cough had improved. EAF can involve the lung and cause symptomatic airway obstruction. For a hilar region mass with imaging characteristics similar to those of lung cancer, a differential diagnosis must be considered.

Eosinophilic angiocentric fibrosis of nasal septum: a case report

Eosinophilic Angiocentric Fibrosis (EAF) is an uncommon benign condition involving the sinonasal tract and rarely the larynx and orbit. Its etiology still remains unclear, although some studies have hypothesized it as a form of IgG4-related systemic disease. Histologically EAF is characterised by the presence of mixed inflammatory cells with eosinophil predominance in the early stage, followed by concentric layers of sclerosis around the small caliber vessels in the late stage. The rarity of this condition, tumor like presentation and oblivious etiology, makes it an interesting entity. We hereby report one such rare case of EAF, since the awareness of this entity is essential to make an accurate diagnosis.

Unexplained Persistent Dyspnea in a Young Woman With Eosinophilic Angiocentric Fibrosis

Eosinophilic angiocentric fibrosis (EAF) is a rare inflammatory disease that primarily involves the nose and sinuses. Involvement of the eye orbit and larynx has also been described. However, it is very rare for this disease to involve the lower respiratory tract and cause dyspnea. We describe a rare case of EAF involving the lower respiratory tract with airway narrowing. A 29-year-old female with a 7-year history of nasal obstruction presented with unexplained persistent dyspnea. EAF was diagnosed via endoscopic biopsy of an irregular mucosal lesion in the posterior wall of the right maxillary sinus. Chest computed tomography and bronchoscopy showed a diffuse inflammatory narrowing of the airway in the tracheobronchial trees. EAF can affect lower respiratory tracts with airway narrowing that can be characterized by dyspnea. We must consider narrowing of the lower respiratory tracts in patients with EAF complaining of unexplained persistent dyspnea.

Primary orbital eosinophilic angiocentric fibrosis with intranasal extension

Eosinophilic angiocentric fibrosis is a chronic, idiopathic disorder that usually involves the upper respiratory tract and features progressive submucosal perivascular fibrosis of unknown etiology. To our knowledge, only 5 cases of eosinophilic angiocentric fibrosis with primary orbital involvement have been reported. We report the case of a 46-year-old man with right proptosis and lateral globe displacement caused by a primary eosinophilic angiocentric fibrosis extending from the orbit into the anterior ethmoid. The nasal extension of the lesion helped in establishing the correct diagnosis. Physicians involved in the treatment of orbital pathologies should be familiar with this entity, because it may manifest as an intraorbital mass growing primarily or secondly into the orbit. The clinical manifestations of eosinophilic angiocentric fibrosis with orbital involvement often mimic other more common ophthalmological diseases. Biopsies are necessary for diagnosis and treatment planning, although cures are usually of palliative effect.

43. A case of eosinophilic angiocentric fibrosis with IGG4-positive plasma cells

Case report Eosinophilic angiocentric fibrosis is a rare fibrotic lesion of the sinonasal and upper respiratory tract. We present a case of eosinophilic angiocentric fibrosis of the maxillary and sphenoid sinuses with infiltration by IgG4-positive plasma cells, raising the possibility of a relationship with IgG4-related sclerosing disease. A 34-year-old female with an unremarkable past medical history presented with a long history of left sided facial pain and congestion unresponsive to corticosteroid therapy. Serum IgG and serum IgG4 levels were not performed. Preoperative CT scans revealed a mass in the left maxillary and sphenoid sinuses. Biopsy of the mass showed fragments of dense fibrous tissue with a prominent concentric arrangement around blood vessels. There was a mild to moderate inflammatory infiltrate including lymphocytes, plasma cells and variable numbers of eosinophils. Isolated blood vessels showed vasculitis with leukocytoclasis. There was no evidence of granulomas, fungi or amyloid. The histological features were consistent with eosinophilic angiocentric fibrosis. Immunohistochemical stain for IgG4 showed the presence of numerous IgG4-positive plasma cells, raising the possibility of a link with IgG4-related sclerosing disease. IgG4-related sclerosing disease is a chronic inflammatory condition in which numerous IgG4 positive plasma cells are seen on immunohistochemical staining and is associated with elevation of serum IgG and IgG4 levels. Since it was first described in the pancreas (autoimmune pancreatitis) in 2001, IgG4-related sclerosing disease has been reported at many sites. As yet, it has not been reported in the sinonasal tract.

Sinonasal Eosinophilic Angiocentric Fibrosis: A Report of Four Cases and Review of Literature

Eosinophilic angiocentric fibrosis (EAF) is a rare, benign condition of unknown etiology involving the sinonasal tract and the upper respiratory airways, and rarely, larynx, and orbit. We report four cases of EAF identified, in three women and one man, aged 31, 57, 27, and 51 years, respectively. The patients complained of sinonasal obstructive symptoms of long duration, nasal masses, epiphora, and/or proptosis. Histologically, all cases demonstrated a dense fibrotic stroma with a perivascular "onion-skin" whorling pattern, and a dense inflammatory infiltrate consisting of lymphocytes, plasma cells, eosinophils, and some neutrophils. In addition, one patient demonstrated modest acute neutrophilic inflammation with focal endothelial proliferation. No patient exhibited clinical or histological evidence of Wegener's granulomatosis, granuloma faciale, Kimura's disease, and malignant lymphomas. Surgical excision was performed in all cases, and to date, medical therapy has been of limited help. The clinical and histopathological features and differential diagnoses of this underreported EAF condition are discussed.

Eosinophilic angiocentric fibrosis of the sinonasal tract

Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory fibrosing lesion of the upper respiratory tract and orbit that occurs mainly in young to middle-aged women. The etiology of EAF is unknown. To our knowledge, approximately 28 cases have been previously reported in the English literature. We report here 3 additional cases of EAF of the sinonasal tract; 2 in women aged 19 and 31 years, and 1 in a man aged 49 years. The 19-year-old woman is the youngest patient with EAF ever described. The patients presented with a nasal cavity mass, face pain, or nasal obstructive symptoms of long duration.

Atypical eosinophilic angiocentric fibrosis on nasal septum

Eosinophilic angiocentric fibrosis (EAF) is a rare benign condition of unknown aetiology and is most commonly found in the nasal septum and sinus mucosa. We report a case of EAF and present a review of the available literature. A 51-year-old man with progressive nasal obstruction was referred to our hospital. CT and MRI scans revealed a mass on the nasal septum; this was surgically excised. Histological analysis of the resected tumour showed an inflammatory infiltrate with a predominance of eosinophils present in fibrous matrix and absence of eosinophilic vasculitis and onion skinning. Histologically, it resembled granuloma faciale. However, our case was considered to be an EAF although eosinophilic vasculitis and onion skinning were not observed. This was because a cutaneous lesion was absent and the lesion was limited to the nasal septal mucosa.

Eosinophilic Angiocentric Fibrosis of the Sinonasal Tract in a Male Patient with Chronic Bowel Inflammation

Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory fibrosing lesion involving the upper respiratory mucosa, occurring mainly in young to middle aged women (female/male ratio = 2:1). The etiology is unknown; however, severalfactors might play a role in the development of EAF. Among them are prior nasal trauma (in most of the reported cases nasal surgery had been performed afew years prior to diagnosis) and inflammatory or autoimmune etiology (suggested by the ratio and the fact that in many of the reported cases a history of nonspecific allergy was found). We report the first case of EAF affecting a male patient who also suffered from chronic inflammatory bowel disease and rheumatic fever. The patient underwent a diagnostic biopsy of his nasal lesion via an open rhinoplasty approach, with the resulting diagnosis of EAF. Despite the fact that the literature does not show advantages to any specific therapy, the patient elected to remain under observation. During a two-year follow-up period, there is no evidence of progression of disease. The presence of concomitant rheumatoid arthritis and chronic inflammatory bowel disease in our patient, as well as the fact that nine previously reported cases of EAF had allergic/immune symptoms, raise the possibility that inflammatory or autoimmune factors may have a role in the development of this unusual pathological entity.

Nasal fibrosis: long-term follow up of four cases of eosinophilic angiocentric fibrosis

Eosinophilic angiocentric fibrosis is a rare, benign cause of submucosal thickening and fibrosis within the upper respiratory tract. It predominantly affects the nose although cases have been reported in the subglottis. We describe four cases of the disease centred around the nasal cavity, with widespread infiltration of the facial soft tissues and orbit in three of the four patients. Each underwent long term follow up. Multiple surgical resections were required with two of our patients and, to date, medical therapy has been of limited help. The disease process, with its clinical and characteristic histopathological findings, is described. We also discuss the management of the disease following a comprehensive review of, and comparison with, the few prior reported cases.

Massive osteolysis with histologic features of eosinophilic angiocentric fibrosis

Background Massive osteolysis (MO) is a rare destructive process of unknown etiology, first described by Gorham and Stout in 1954. This condition is exceedingly rare with approximately 150 cases described in the literature and 20% involving the jaws and skull. The disease is characterized by a proliferation of thin-walled vasculature channels and inflammation with regional osteolysis. Eosinophilic angiocentric fibrosis (EAF) is a newly described entity also of unknown etiology. Previously reported cases of EAF have been restricted to the nose and upper airway tract. The histology of EAF is characterized by a perivascular fibrosis and associated tissue eosinophilia. A 16-year-old girl with MO affecting the right mandible and bilateral maxilla and histologic features of EAF is described. Objective We attempt to determine whether similarities exist between MO and EAF and if these 2 entities can be distinguished based on the clinical, radiographic, or histopathologic features. Study design A comprehensive search of the Medline database from 1966 through 2003 and a review of the reference lists of relevant articles of MO and EAF formed the basis of this study. These data were compared to the present case of MO with histologic features of EAF. Results The case report has clinical and radiographic features of MO. The process was progressive, with uncontrolled osteolysis despite aggressive surgery and radiation therapy. The histologic features identified are comparable to those described for EAF. However our literature review could not identify clinical and radiographic similarities between this case and those of EAF reported in the literature. Conclusion The clinical and radiographic features of this case are consistent with a diagnosis of MO, yet the histopathology is more typical of EAF. The relationship between these 2 poorly understood conditions, if any, remains obscure.

An unusual cause of nasal airway obstruction: eosinophilic angiocentric fibrosis

Eosinophilic angiocentric fibrosis is a rare disease of unknown cause. This disease, which involves the upper respiratory mucosa, resembles another rare entity, granuloma faciale. Allergy is thought to be an etiological factor and complete surgical excision is the preferred treatment. A case of eosinophilic angiocentric fibrosis of the nasal septum causing airway obstruction is presented here. This is the sixth case to be reported in the English literature, with well-documented histopathological confirmation.