Benralizumab monotherapy was insufficient to induce remission in patients with active eosinophilic granulomatosis with polyangiitis.

Eosinophils play an important pathogenetic role in the development of eosinophilic granulomatosis with polyangiitis (EGPA). EGPA has long been treated with systemic corticosteroids and immunosuppressive agents. However, in recent years, biologic agents targeting eosinophils (anti-IL-5 antibody; mepolizumab) have also been used. Evidence regarding the effectiveness of using benralizumab, anti-IL-5 receptor α monoclonal antibody that depletes eosinophils via antibody-dependent cell-mediated cytotoxicity, has been growing. Benralizumab is used as a steroid-sparing treatment option for EGPA. Clinical studies have evaluated the effects of using mepolizumab or benralizumab in combination with steroids for the treatment of EGPA. However, to date, there have been no reports of using biologics alone. Herein, we describe the case of a patient with active EGPA refractory to benralizumab monotherapy. The patient achieved significant improvement in symptoms after administration of corticosteroids during hospitalization. Benralizumab monotherapy might not be considered a therapeutic option for patients with active EGPA in whom corticosteroids are initially indicated.

Isolated arteritis misdiagnosed as bilateral orbital tumors in a patient with acute ischemic stroke.

Vasculitis is a heterogeneous group of disorders characterized by multifocal segmental inflammation of the small and medium vessels of the central nervous system. The predominant symptoms of cerebral vasculitis are stroke, headache, and encephalopathy. Additional symptoms include seizures, cranial nerve palsies, and myelopathy. Imaging techniques play a crucial role in identifying the diagnosis of vasculitis and demonstrating brain involvement. An 89-year-old woman with permanent atrial fibrillation developed an embolic stroke. In treatment, intravenous thrombolysis and thrombectomy with complete antegrade reperfusion of the left middle cerebral artery was used, without the clinical effectiveness. Brain MRI revealed bilateral oval lesions in medial parts of the orbits, which were initially misinterpreted as orbital tumors. Final diagnosis confirmed thickened arterial walls as orbital changes due to inflammatory arteritis. Ten days later, follow-up MRI was performed and showed complete regression of the orbital masses. Primary central nervous system vasculitis, manifesting as acute ischemic stroke, may be reversible with early systemic thrombolytic treatment.

Retropharyngeal edema: A rare manifestation of eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by excessive eosinophil accumulation in the peripheral blood and affected tissues with development of granulomatous vasculitic organ damage. Although upper airway and neck involvement is seen in patients with EGPA, retropharyngeal inflammation has never been reported. We report a case of retropharyngeal edema in a 70-year-old woman with EGPA. Her symptoms improved and the retropharyngeal edema disappeared on computed tomography following treatment. EGPA should be considered as a differential diagnosis in patients with asthma presenting with neck swelling and dysphagia.

Diagnosis and treatment of eosinophilic myocarditis.

Eosinophilic myocarditis is a type of inflammatory cardiomyopathy characterized by eosinophilic infiltration into myocardial tissue. The accurate myocarditis incidence rate is difficult to determine because of the clinical limitations of an endomyocardial biopsy. The primary pathogenesis of eosinophilic myocarditis is the release of related substances by eosinophils, leading to cell membrane damage and cell destruction. However, evidence suggests that specific genes play a role in myocarditis development.As CMR imaging availability increases, the diagnosis rate of eosinophilic myocarditis will increase. The diagnosis of myocarditis mainly depends on an endocardial biopsy. Glucocorticoids can relieve patients' symptoms, but the early use of steroids may prevent intermediate disease stage development (i.e., thrombonecrosis and fibrosis with wall thrombosis). Anticoagulant therapy may also affect disease development. In addition to routine follow-up, a regular myocardial biopsy should be considered for discharged patients, if possible.

Myeloperoxidase anti-neutrophil cytoplasmic antibody-associated vasculitis with a unique imaging presentation of organizing pneumonia: A case report.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of blood vessel inflammation diseases of autoimmune origin. Myeloperoxidase (MPO) ANCA is closely related to ANCA associated AAV. The MPO-ANCA positive AAV patients have lung involvement at high rates; however, there are only a few reported cases with organizing pneumonia (OP). A 78-year-old man was presented to our hospital due to a fever of 38 °C despite a whole month of antibiotics treatment. Chest computed tomography image revealed restricted consolidations visible in the middle lobe of the right lung and the upper lobe of the left lung, which suggested an OP pattern. MPO-ANCA and urine occult blood tests were positive. Histopathological examination of the transbronchial biopsy revealed OP and mucus plug. Histological findings on renal biopsy showed necrotizing glomerulonephritis related to AAV. The patient was diagnosed with MPO-ANCA positive AAV and was treated with systemic corticosteroid therapy, from which he recovered rapidly. Thus, when diagnosing OP, the possibility of AAV should be considered by ordering patients' serum ANCA and occult hematuria tests.

An unusual case of eosinophilic lung disease with multiple cyst formation.

We report here an unusual case of eosinophilic necrotizing inflammation of the lung that mimicked chronic eosinophilic pneumonia. A 71-year-old man who lived in an unsanitary environment and was referred to our hospital with suspected pneumonia. Peripheral blood eosinophilia was observed, and computed tomography indicated extensive consolidation with multiple cystic lesions, mainly in the left lung. A histological analysis using video-assisted thoracic surgery revealed diffuse infiltration of inflammatory cells into the alveolar wall and massive accumulation of macrophages and eosinophils in the airspace. Many tiny eosinophilic abscesses were scattered through the tissue. These findings were more severe than those associated with chronic eosinophilic pneumonia. Immunostaining revealed the deposition of eosinophil granular protein and the presence of extracellular traps and Charcot-Leyden crystals, which suggested excessive eosinophil activation. Interestingly, the patient's symptoms and clinical findings gradually improved without treatment after admission. He was discharged to a clean residence and did not have a recurrence for 19 months. The observations suggest a hypersensitivity reaction to an environmental allergen and consequent multiple cyst formation in association with eosinophilic necrotizing inflammation, although further studies are warranted.

Factors that affect blood eosinophil counts in a non-asthmatic population: Post hoc analysis of data from Brazil.

BACKGROUND: Improved understanding of the normal range of blood eosinophil counts (BEC) and conditions that influence them in non-asthmatic individuals should allow more accurate estimation of the threshold at which eosinophilic disease should be considered, diagnosed, and treated. This analysis investigated the impact of atopy, smoking, and parasitic infection on BEC. METHODS: This was a post hoc analysis of non-asthmatic subjects from a case-control study (CONEP 450/10) conducted at the Program for Control of Asthma in Bahia (ProAR). Participant BECs were measured at baseline; correlations between predefined risk factors and BEC were assessed via univariate and stratified analysis. RESULTS: Of the 454 participants included, 3% were helminth parasite-positive, 18% were non-helminth parasite-positive; and 450 had BEC data. The median (interquartile range [IQR]) BEC was 152 (96, 252) cells/µL. Any positive skin prick test, elevated total immunoglobulin E, allergic rhinitis, and being a current smoker were all individually associated with higher BEC (p < 0.05) compared with BEC in participants without these factors, but having a non-helminthic parasitic infection was not. Participants with all 4 risk factors that were associated with higher BEC had a median (IQR) BEC of 192 cells/µL (94, 416) versus 106 cells/µL (70, 164) for those with no risk factors. CONCLUSIONS: In non-asthmatic subjects, atopy, allergic rhinitis, and current smoking status were associated with higher BEC compared with subjects without these factors, but BEC values were well below the threshold commonly accepted as normal. Therefore, BEC should be interpreted in the context of an individual's medical conditions and other BEC-influencing factors.

Allergy and the Heart: Eosinophilic Myocarditis With Biventricular Thrombi.

A 40-year-old woman with history of atopy and peripheral eosinophilia presented with clinical signs of heart failure. Echocardiography revealed a restrictive cardiomyopathy with biventricular thrombi. Hypereosinophilic syndrome resulting in eosinophilic myocarditis (Loeffler's syndrome) was diagnosed. This case highlights the workup, diagnosis, and management of hypereosinophilic syndrome with eosinophilic myocarditis. (Level of Difficulty: Advanced.).

Fulminant Eosinophilic Myocarditis: A Rare and Life-Threatening Presentation of Eosinophilic Granulomatosis With Polyangiitis.

We describe a case of fulminant eosinophilic myocarditis as the first presentation of eosinophilic granulomatosis with polyangiitis, promptly managed with extracorporeal membrane oxygenation. This case highlights the multidisciplinary work involving all health care professionals in the acute management of these patients and discusses it from an educational point of view. (Level of Difficulty: Intermediate.).

Eosinophilic Granulomatosis With Polyangiitis Presenting as an Acute Coronary Syndrome.

Eosinophilic granulomatosis with polyangiitis, formerly Churg-Strauss Syndrome, is an uncommon disorder that carries a high mortality when coronary artery disease develops. Early recognition and treatment is crucial. We highlight an unusual presentation of acute coronary syndrome not associated with atherosclerotic coronary disease. (Level of Difficulty: Intermediate.).

Flare of eosinophilic granulomatosis with polyangiitis related to pregnancy: Case report and review of the literature.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by excessive eosinophil accumulation in the peripheral blood and affected tissues with development of granulomatous vasculitic organ damage. It is strongly associated with asthma and ear-nose-throat disease. It often affects patients between the ages of 40 and 60 years. It is unknown whether pregnancy impacts the disease activity of EGPA, including initial diagnosis or relapse. Because of its rarity and age of susceptibility, there are few reported cases describing pregnancy in women with quiescent or active EGPA. Here, we describe a young woman who experienced EGPA relapse during pregnancy and subsequently underwent an elective caesarean section for non-reassuring fetal status at 37 weeks without complication.

Tocilizumab-effective multicentric Castleman's disease with infiltration of eosinophil and IgG4-positive plasma cells: A case report.

A 67-year-old woman with fever and cough was diagnosed with eosinophilic pneumonia because of eosinophilia and increased eosinophil levels in the bronchoalveolar lavage fluid and transbronchial biopsy lung specimens. However, prednisolone therapy at a previous hospital was ineffective. Histological findings from thoracoscopic lung and lymph node biopsies were consistent with multicentric Castleman's disease (MCD). Since specimens also showed prominent eosinophil and IgG4-positive plasma cell infiltration, it was difficult to distinguish IgG4-related disease (IgG4-RD) from MCD. Administration of prednisolone plus tocilizumab improved the symptoms and lung lesions, and prednisolone administration was successfully reduced and then terminated. The present case highlights the difficulty in diagnosing MCD and IgG4-RD, and suggests that combined administration of tocilizumab and prednisolone might be effective in such a case.

Generalized morphea/eosinophilic fasciitis overlap after epoxy exposure.

Generalized morphea is associated with epoxy resin vapors and is characterized by the development of lesions shortly after exposure. Morphea presenting along with eosinophilic fasciitis (EF), or morphea/EF overlap, is rare and an indicator of poor prognosis and resistance to treatment. Here we present a case of generalized morphea/EF overlap linked to epoxy exposure. Our patient received multiple therapies-ultraviolet A1 phototherapy, prednisone, methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporine, and rituximab-none of which led to a significant response. The refractory nature of this disease warrants vigilance in its association with epoxy exposure.

Interferon-α induced remission in three patients with eosinophilic granulomatosis and polyangiitis. A case study.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small vessel vasculitis associated with asthma and eosinophilia. Optimal therapy for maintenance of remission is yet to be defined. We present a case-series of three patients with EGPA in whom IFN-α, an immunomodulatory cytokine induced remission, which was maintained even after discontinuation of the drug. In all patients (ages 60, 51, and 50 years), remission was associated with normalisation of eosinophil counts and IgE-levels. Moreover, the patients remained in remission for one to four years. Two patients did not need further immunosuppression, one patient required low dose maintenance therapy. Although reversible side effects occur, IFN-α-therapy induces long-term remission of EGPA even after discontinuation of treatment.