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Journal articles on the topic 'Eosinophilic granulomatosis with polyangiitis'

Author: Grafiati

Published: 10 March 2023

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1

Trivioli, Giorgio, Benjamin Terrier, and Augusto Vaglio. "Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management." Rheumatology 59, Supplement_3 (April 29, 2020): iii84—iii94. http://dx.doi.org/10.1093/rheumatology/kez570.

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Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cells and eosinophils. Eosinophilic granulomatosis with polyangiitis must be differentiated from several conditions, including hypereosinophilic syndromes and other small-vessel vasculitides. The overall survival is good; however, patients frequently relapse and have persistent symptoms. The recently developed monoclonal antibodies targeting B cells and eosinophilopoietic cytokines such as IL-5 are emerging as valid alternatives to conventional immunosuppressive therapies. In this review, we discuss the essential features of eosinophilic granulomatosis with polyangiitis, with particular respect to the most relevant issues concerning clinical presentation and management.

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Ito, Hiroyuki, Yusuke Mishima, Tsubomi Cho, Naoki Ogiwara, Yoshimasa Shinma, Masashi Yokota, Kazuya Anzai, et al. "Eosinophilic Cholecystitis Associated with Eosinophilic Granulomatosis with Polyangiitis." Case Reports in Gastroenterology 14, no. 3 (December 11, 2020): 668–74. http://dx.doi.org/10.1159/000511863.

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We report a case of eosinophilic cholecystitis associated with eosinophilic granulomatosis with polyangiitis (EGPA) complicated by cerebral hemorrhage. A 60-year-old man presented to a local hospital with a diagnosis of acute cholecystitis, with persistent fever and epigastric pain for 2 weeks. His symptoms persisted despite 3-week hospitalization; therefore, he was transferred to our hospital for further evaluation. Laboratory investigations upon admission showed white blood cells 26,300/µL and significant eosinophilia (eosinophils 61%). Abdominal computed tomography revealed no gallbladder enlargement but a circumferentially edematous gallbladder wall. Additional blood test results were negative for antineutrophil cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies; however, immunoglobulin (Ig)G and IgE levels were high at 1,953 mg/dL and 3,040/IU/mL, respectively. He improved following endoscopic transnasal gallbladder drainage for cholecystitis and was diagnosed with EGPA and received corticosteroid and immunosuppressant combination therapy. The eosinophil count decreased immediately after treatment, and abdominal pain and numbness resolved. He returned with left-sided suboccipital hemorrhage likely attributed to EGPA 6 months after discharge. EGPA is characterized by inflammation of small blood vessels and clinically manifests with an allergic presentation of bronchial asthma, as well as renal dysfunction, interstitial pneumonia, enteritis, and cerebral hemorrhage. Few reports have described cholecystitis as a presenting symptom of EGPA. We report a rare case of such a presentation with added considerations.

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Ogurtsova, Alina Vadimovna, Ekaterina Sergeevna Kosarenko, Tayiana Valeryrvna Zuevskaya, and Evgenia Nikolaevna Ivanova. "CLINICAL CASE OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS." Scientific medical Bulletin of Ugra 33, no. 3 (2022): 44–50. http://dx.doi.org/10.25017/2306-1367-2022-33-3-44-50.

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Eosinophilic granulomatosis with polyangiitis (Churg – Strauss granulomatosis) is a rare autoimmune disease characterized by eosinophilic, granulomatous inflammation in combination with necrotizing vasculitis, predominantly affecting small and medium-sized vessels and most often manifested by damage to the respiratory tract. The nonspecificity of symptoms of eosinophilic granulomatosis at the onset of the disease and the similarity of symptoms with other vasculitis, systemic, infectious and neoplastic diseases makes it difficult to make a diagnosis. The paper presents a clinical case demonstrating the difficulties of differential diagnosis of eosinophilic granulomatosis with polyangiitis with other systemic diseases.

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Pavan, Matteo, Anna Agrusti, Andrea Trombetta, Serena Pastore, Alberto Tommasini, Valentina Moressa, Federico marchetti, Andrea Taddio, and Alessandro Ventura. "La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore." Medico e Bambino 39, no. 9 (November 9, 2020): 569–74. http://dx.doi.org/10.53126/meb39562.

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Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

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5

Kavanagh, F. G., W. Hasan, D. A. Smyth, and J. E. Fenton. "Polyps, grommets and eosinophilic granulomatosis with polyangiitis." Journal of Laryngology & Otology 132, no. 3 (January 9, 2018): 236–39. http://dx.doi.org/10.1017/s0022215117002444.

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AbstractObjective:To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.Methods:A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis.Results:Sixteen patients (10 males and 6 females) were identified. The mean age of grommet insertion was 45.4 years. The mean number of grommets inserted per patient was 1.6. The mean number of nasal polypectomies was 1.7. All 16 patients had paranasal sinus abnormalities and otitis media with effusion, 14 had asthma, 9 had serological eosinophilia and 7 had extravascular eosinophilia. Nine patients met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis.Conclusion:The co-presence of nasal polyps and resistant otitis media with effusion should raise the possibility of eosinophilic granulomatosis with polyangiitis.

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6

Seccia, V., L. Cristofani-Mencacci, I. Dallan, S. Fortunato, M. L. Bartoli, S. Sellari-Franceschini, M. Latorre, P. L. Paggiaro, and C. Baldini. "Eosinophilic granulomatosis with polyangiitis and laryngeal involvement: review of the literature and a cross-sectional prospective experience." Journal of Laryngology & Otology 132, no. 7 (June 11, 2018): 619–23. http://dx.doi.org/10.1017/s0022215118000737.

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AbstractBackgroundEosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis show variable otorhinolaryngological involvement. Up to 14 per cent of granulomatosis with polyangiitis patients have subglottis involvement; little is known about the laryngeal involvement in eosinophilic granulomatosis with polyangiitis.MethodA literature review was conducted, together with a prospective cross-sectional analysis of 43 eosinophilic granulomatosis with polyangiitis patients. All patients underwent fibre-optic laryngoscopy with narrow-band imaging, and completed health-related questionnaires.ResultsThe literature review showed only two cases of laryngeal involvement in eosinophilic granulomatosis with polyangiitis; in our cohort, no cases of subglottis stenosis were found, but local signs of laryngeal inflammation were present in 72 per cent of cases. Of the patients, 16.2 per cent had a pathological Reflux Finding Score (of 7 or higher).ConclusionLaryngeal inflammation in eosinophilic granulomatosis with polyangiitis is frequent. It is possibly due more to local factors than to eosinophilic granulomatosis with polyangiitis itself. However, ENT evaluation is needed to rule out possible subglottis inflammation. These findings are in line with current literature and worthy of confirmation in larger cohorts.

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Ou, Changxing, Jianjuan Ma, Ning Lai, You Li, Jiaxing Xie, Xueyan Zhang, and Qingling Zhang. "Severe asthma with viral infection can develop into eosinophilic granulomatosis with polyangiitis." Rheumatology and Immunology Research 2, no. 4 (December 1, 2021): 249–54. http://dx.doi.org/10.2478/rir-2021-0034.

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Abstract Asthma is common in eosinophilic granulomatosis with polyangiitis (EGPA), and the annual incidence of EGPA in patients with asthma is much higher compared with the general population, and the trigger factor for this is unknown. We report a case of a 19-year-old male with a background of severe asthma who presented with eosinophilic lung infiltration after viral infection, which progressed to clinical EGPA. The diagnosis of EGPA was supported by an initial clinical presentation of recurrent cough and wheezing accompanied by a red rash, followed by peripheral eosinophilia, a high eosinophil percentage in bronchoalveolar lavage fluid (BALF), and migratory pulmonary eosinophilic infiltrates. Lung biopsy showed blood vessels with extravascular eosinophils. The patient responded well to high-dose glucocorticoids and cyclophosphamide, and symptoms and biochemical markers improved. Our literature review identified few reports on the triggers of EGPA, which highlights that viral infection may be a risk factor for asthma that progresses to EGPA.

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8

Matsumoto, Yoshinori. "Eosinophilic granulomatosis with polyangiitis." Okayama Igakkai Zasshi (Journal of Okayama Medical Association) 134, no. 2 (August 1, 2022): 95–98. http://dx.doi.org/10.4044/joma.134.95.

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9

Shukla, Ratnakar, Ashwini Kandikatla, NitinJ Nadkarni, and Anant Patil. "Eosinophilic granulomatosis with polyangiitis." International Journal of Applied and Basic Medical Research 12, no. 2 (2022): 148. http://dx.doi.org/10.4103/ijabmr.ijabmr_528_21.

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10

Korman, Abraham M., and Catherine G. Chung. "Eosinophilic Granulomatosis With Polyangiitis." JAMA Dermatology 157, no. 6 (June 1, 2021): 722. http://dx.doi.org/10.1001/jamadermatol.2020.2279.

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Yong, Geng-Yi, and Amelia L. S. Lim. "Eosinophilic Granulomatosis with Polyangiitis." New England Journal of Medicine 386, no. 4 (January 27, 2022): 374. http://dx.doi.org/10.1056/nejmicm2112515.

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12

Berti, Alvise, Divi Cornec, Marta Casal Moura, Robert J. Smyth, Lorenzo Dagna, Ulrich Specks, and Karina A. Keogh. "Eosinophilic Granulomatosis With Polyangiitis." Chest 157, no. 5 (May 2020): 1086–99. http://dx.doi.org/10.1016/j.chest.2019.11.045.

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13

Boudreaux, BL, A. Perez, S. Bajracharya, S. Barry, R. Mohandas, and EA Aguilar. "Eosinophilic granulomatosis with polyangiitis." American Journal of the Medical Sciences 365 (February 2023): S263—S264. http://dx.doi.org/10.1016/s0002-9629(23)00495-0.

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Djukanovic, Ratko, and Paul M. O’Byrne. "Targeting Eosinophils in Eosinophilic Granulomatosis with Polyangiitis." New England Journal of Medicine 376, no. 20 (May 18, 2017): 1985–86. http://dx.doi.org/10.1056/nejme1704402.

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15

Rasheed, Nidaa, Rahul Ahuja, and Hena Borneo. "Polyangiitis overlap syndrome: a novel presentation of microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis." BMJ Case Reports 14, no. 2 (February 2021): e239031. http://dx.doi.org/10.1136/bcr-2020-239031.

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Polyangiitis overlap syndrome (POS) is a diagnostic term coined by Leavitt and Fauci that characterises patients with overlapping features of more than one vasculitis. Prior case studies of antineutrophil cytoplasmic antibodies (ANCA)-associated POS have only been published in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis alongside proteinase-3/cytoplasmic (C)-ANCA positivity. We present a case of a 60-year-old woman with dyspnoea, hemoptysis, positive perinuclear-ANCA and renal biopsy demonstrating evidence of microscopic polyangiitis. In addition, our patient also had asthma, mononeuritis multiplex, eosinophilia and migratory pulmonary infiltrates, thus fulfilling the criteria for EGPA. This novel case report suggests that POS is not limited to C-ANCA positivity and has variable presentations.

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Hattori, Keita, Yuri Teramachi, Yoshinori Kobayashi, Takeshi Ito, Takatoshi Morinaga, Hirohumi Tamai, and Yoshihiro Yamamoto. "A Case of Effective Mepolizumab Induction Therapy for Severe Eosinophilic Granulomatosis with Polyangiitis Diagnosed by Eosinophilic Cholecystitis and Interstitial Nephritis." Case Reports in Rheumatology 2021 (June 19, 2021): 1–5. http://dx.doi.org/10.1155/2021/6678893.

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A 66-year-old man with a history of bronchial asthma and sinusitis was admitted with cholecystitis and peripheral neuropathy. The histopathological findings of the gallbladder revealed necrotic vasculitis and granulomatous inflammation with marked eosinophilic infiltration. Kidney biopsy also showed marked eosinophilic infiltration in the tubulointerstitial area and eosinophilic tubulitis. He was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) and treated with corticosteroids. However, he showed no response. Therefore, he was administered mepolizumab 300 mg, which resulted in clinical improvement, including normalization of the eosinophil and CRP levels. We herein describe the first case of successful induction therapy of EGPA using mepolizumab.

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RAZIQ, FAZAL I. "EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS WITHOUT ANY PERIPHERAL EOSINOPHILIA." Chest 162, no. 4 (October 2022): A1772. http://dx.doi.org/10.1016/j.chest.2022.08.1475.

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18

Karadeniz, Hazan, and Abdurrahman Tufan. "Eosinophilic Granulomatosis with Polyangiitis (EGPA)." Acta Medica 52 (April 30, 2021): 39. http://dx.doi.org/10.32552/2021.actamedica.604.

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Al Arfaj, Abdurhman S., Mohammad Al Anazi, Najma Khalil, Akbar Ali Khan Pathan, and Narsimha Reddy Parine. "Familial Eosinophilic Granulomatosis with Polyangiitis." Open Journal of Rheumatology and Autoimmune Diseases 07, no. 03 (2017): 137–46. http://dx.doi.org/10.4236/ojra.2017.73013.

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20

Kawashima, Kayoko. "Eosinophilic granulomatosis with polyangiitis: EGPA." Journal of Japan Society of Immunology & Allergology in Otolaryngology 36, no. 4 (2018): 275–78. http://dx.doi.org/10.5648/jjiao.36.275.

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21

Nakajima, Hiroshi. "4) Eosinophilic Granulomatosis with Polyangiitis." Nihon Naika Gakkai Zasshi 106, Suppl (February 20, 2017): 112a. http://dx.doi.org/10.2169/naika.106.112a.

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Nakajima, Hiroshi. "4) Eosinophilic Granulomatosis with Polyangiitis." Nihon Naika Gakkai Zasshi 106, no. 9 (September 10, 2017): 1832–37. http://dx.doi.org/10.2169/naika.106.1832.

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Ohshimo, Shinichiro, Josune Guzman, Ulrich Costabel, and Francesco Bonella. "Differential diagnosis of granulomatous lung disease: clues and pitfalls." European Respiratory Review 26, no. 145 (August 9, 2017): 170012. http://dx.doi.org/10.1183/16000617.0012-2017.

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Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis.

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Tulbă, Delia, Răzvan Adrian Ionescu, Daniela Gologanu, Marius Ioan Balea, and Simona Caraiola. "Eosinophilic Granulomatosis with Polyangiitis: Diagnostic Dilemmas." Internal Medicine 15, no. 1 (March 1, 2018): 63–68. http://dx.doi.org/10.2478/inmed-2018-0007.

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AbstractIntroduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a systemic vasculitis of the small vessels that often associates asthma and blood/tissue eosinophilia.Case presentation. A 58-year-old woman was admitted to our hospital for progressive exertional dyspnea. She had been diagnosed with asthma three years earlier. Recent multiple thoracic computed tomography scans displayed non-fixed interstitial lung abnormalities, whereas the infectious workup (HIV, parasites) was negative.On admission, the clinical examination noted prolonged expiratory phase. Paraclinical tests revealed biological inflammatory syndrome, eosinophilia, polyclonal hypergammaglobulinemia, elevated total IgE level, negative anti-neutrophil cytoplasmic antibodies and proteinuria of nephritic pattern.The pulmonary evaluation reconfirmed the obstructive ventilatory dysfunction and interstitial lung abnormalities - interlobular septal thickening and diffuse ground-glass opacification. The histopathological examination of a transbronchial biopsy specimen identified leukocytoclastic necrotizing vasculitis and tissue eosinophilia.Provided the clinical and paraclinical setting, specifically the asthma, blood and extravascular eosinophilia, paranasal sinus abnormalities, non-fixed pulmonary infiltrates and the histopathologically confirmed necrotizing vasculitis, the diagnosis of EGPA seemed appropriate.Conclusion. In the absence of diagnostic criteria, EGPA diagnosis is often challenging. Although certain clinical and imaging features could assist the diagnosis, biopsy remains the diagnostic gold standard.In the setting of lung involvement, open lung biopsy is usually required for EGPA histopathological proof, but few cases diagnosed by transbronchial biopsy have been reported. This method often identifies discrete, nonspecific lesions or an incomplete spectrum of pathognomonic abnormalities but has the advantage of minimal invasivity that justifies its use as an alternative diagnostic technique.

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Rimmer, J., P. Andrews, and V. J. Lund. "Eosinophilic angiocentric fibrosis of the nose and sinuses." Journal of Laryngology & Otology 128, no. 12 (November 17, 2014): 1071–77. http://dx.doi.org/10.1017/s0022215114002552.

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AbstractBackground:Eosinophilic angiocentric fibrosis is a rare benign disorder of the upper respiratory tract. It is slow growing and progressive, with characteristic histological appearances.Methods:We report the largest single-institution case series of sinonasal eosinophilic angiocentric fibrosis to date, comprising nine patients. The current literature is reviewed, showing emerging evidence that this condition may belong to the immunoglobulin G4-related disease spectrum.Results:The series comprised five female and four male patients, with a mean age at presentation of 53 years. All were treated surgically. Six patients had no signs of recurrent disease after an average of 8.5 years. One patient went on to develop granulomatosis with polyangiitis (Wegener's granulomatosis), which required immunosuppressive therapy.Conclusion:The first-line management of this rare condition is complete surgical excision. Chronic granulomatous conditions, including granulomatosis with polyangiitis, should be excluded before a diagnosis is made, and patients should be carefully followed.

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Mazurov, V. I., I. B. Belyaeva, A. L. Chudinov, and O. V. Inamova. "Complications and causes of mortality in patients with primary necrotizing systemic vasculitis." Medical alphabet, no. 33 (December 13, 2021): 30–34. http://dx.doi.org/10.33667/2078-5631-2021-33-30-34.

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The article reviews the clinical symptoms, main complications in patients with ANCA-associated systemic vasculitis (granulomatous with polyangiitis, microscopic polyangiitis, eosinophilic granulomatous with polyangiitis) and polyarteritis nodosa during the first three years of the disease. We evaluated the relationship between complications and long-term glucocorticosteroid therapy. Patients with granulomatosis had the most unfavourable course of the disease with a high risk of complications. A high incidence of ischemic strokes and chronic kidney disease in patients with microscopic polyangiitis has been identified. We also analyzed cases of lethal outcomes in patients with primary systemic vasculitis.

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Cottin, Vincent, Elisabeth Bel, Paolo Bottero, Klaus Dalhoff, Marc Humbert, Romain Lazor, Renato A. Sinico, et al. "Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg–Strauss)." European Respiratory Journal 48, no. 5 (September 1, 2016): 1429–41. http://dx.doi.org/10.1183/13993003.00097-2016.

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The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.4±14.1 years), with a mean±sd blood eosinophil count of 7.4±6.4×109 L−1 at diagnosis. There was a mean±sd of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corticosteroid treatment, the severity of asthma increased 3–6 months before the onset of the systemic manifestations. Asthma was severe in 57%, 48%, and 56% of patients at diagnosis, at 3 years, and at the final visit, respectively. Persistent airflow obstruction was present in 38%, 30%, and 46% at diagnosis, at 3 years, and at the final visit, respectively.In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long-term management and morbidity.

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Ekeigwe, Ngozi Lina, Olufemi Adelowo, Ehiaghe Lonia Anaba, and Hakeem Olaosebikan. "Eosinophilic granulomatosis with polyangiitis in a Nigerian woman." BMJ Case Reports 12, no. 6 (June 2019): e228901. http://dx.doi.org/10.1136/bcr-2018-228901.

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Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. There were also associated symptoms of peripheral neuropathy, weight loss, peripheral blood eosinophilia and positive perinuclear ANCA and she was previously managed for rheumatoid arthritis and asthma. She fulfilled the American College of Rheumatology criteria for EGPA and was treated with glucocorticoids and methotrexate which led to significant improvement of her symptoms.

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Isozaki, Takeo, Tetsuya Homma, Hironori Sagara, and Tsuyoshi Kasama. "Role of Cytokines in EGPA and the Possibility of Treatment with an Anti-IL-5 Antibody." Journal of Clinical Medicine 9, no. 12 (November 30, 2020): 3890. http://dx.doi.org/10.3390/jcm9123890.

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of systemic vasculitis with eosinophilia in the peripheral blood, which is preceded by bronchial asthma or allergic disease. EGPA is pathologically characterized by microangiopathy granulomatosis vasculitis. Vasculitis can be exacerbated and cause central nervous system and cardiovascular disorders and gastrointestinal perforation. Histological examination reveals eosinophil infiltration and granulomas in lesions in areas such as the lung, nervous system, and skin. Laboratory tests show inflammatory findings such as C-reactive protein (CRP) elevation, increased eosinophils, elevated serum IgE, and elevated myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA). MPO-ANCA is positive in approximately 40–70% of cases of this disease. EGPA is a necrotizing vasculitis that affects small- and medium-sized blood vessels; however, it differs from other types of ANCA-related vasculitis (such as microscopic polyangiitis and granulomatosis) because it is preceded by bronchial asthma and eosinophilia in the blood and tissues. Treatment with immunosuppressive agents such as steroids or cyclophosphamide depends on the Five Factor Score, which predicts the prognosis and severity of the condition. If the effect of appropriate treatment with steroids is insufficient, the anti-interleukin-5 antibody mepolizumab can be administered. The combination of mepolizumab with standard treatment leads to a significantly longer duration of remission, a higher proportion of patients who achieve sustained remission, and less steroid use than with a placebo.

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Gundappa, Chandramohan, Sujit Surendran, Arun Gandhi, AnilaAbraham Kurien, and Edwin Fernando. "An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis." Saudi Journal of Kidney Diseases and Transplantation 28, no. 3 (2017): 639. http://dx.doi.org/10.4103/1319-2442.206450.

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Mekouar, Fadwa, Naoual Elomri, Mohamed Jira, Naoufal Assoufi, Mohamed Qatni, and Jamal Fatihi. "GRANULOMATOSE EOSINOPHILIQUE AVEC POLYANGEITE: PRESENTATIONS INHABITUELLES. EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: UNUSUAL PRESENTATIONS." International Journal of Advanced Research 9, no. 10 (October 31, 2021): 1199–203. http://dx.doi.org/10.21474/ijar01/13667.

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Introduction:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown etiology, affecting small and medium-sized vessels and associating asthma,pulmonary involvement, peripheral eosinophilia and extravascular eosinophilic granulomas. We report three GEPA sightings of unusual revelations. Methods:These are three cases of GEPA from the internal medicine department B, Mohammed V military hospital in Rabat. Results:Three patients aged between 27 and 70 years. The most frequently found manifestations were pulmonary, cutaneous, neurological and digestive. All patients presented with peripheral eosinophilia. The ANCA were negative. All patients were treated with corticosteroids in one case, immunosuppressive therapy were used. Conclusion:In this article, GEPA was revealed by an acute surgical abdomen (appendicitis) in one case, a febrile rash in the second and liver damage in the third patient. Our patients responded well to corticosteroid therapy but the treatment must be adapted depending on the case.

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Fennelly, Evelyn, Emily Greenan, and Conor C. Murphy. "Corneal melt secondary to eosinophilic granulomatosis with polyangiitis." BMJ Case Reports 12, no. 6 (June 2019): e229859. http://dx.doi.org/10.1136/bcr-2019-229859.

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis that can affect any organ system. It is most often characterised by chronic airway inflammation along with prominent peripheral blood eosinophilia, although the disease can affect the cardiovascular, gastrointestinal, renal or central nervous systems. Ocular manifestations are uncommon and when they do occur, are varied in their clinical presentations. To the best of our knowledge, this is the first case of corneal melt secondary to EGPA to have been reported.

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Özdemir, Bahar, Abdulsamet Erden, Serdar Can Güven, Berkan Armagan, Hakan Apaydin, Özlem Karakas, Ahmet Gokhan Akdag, İhsan Ates, Orhan Kucuksahin, and Ahmet Omma. "COVID-19 and eosinophilic granulomatosis with polyangiitis or COVID-19 mimicking eosinophilic granulomatosis with polyangiitis?" Rheumatology International 41, no. 8 (May 25, 2021): 1515–21. http://dx.doi.org/10.1007/s00296-021-04896-2.

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Wang, Chrong-Reen, Yi-Shan Tsai, Hung-Wen Tsai, and Cheng-Han Lee. "B-Cell-Depleting Therapy Improves Myocarditis in Seronegative Eosinophilic Granulomatosis with Polyangiitis." Journal of Clinical Medicine 10, no. 19 (October 2, 2021): 4577. http://dx.doi.org/10.3390/jcm10194577.

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Cardiac involvement is a major mortality cause in eosinophilic granulomatosis with polyangiitis (EGPA), requiring novel therapeutics to spare the use of cyclophosphamide with known cardiotoxicity. Despite the observed efficacy of B-cell-depleting therapy in myocarditis of seropositive microscopic polyangiitis, it remains to be elucidated in seronegative EGPA. A retrospective study was performed in 21 hospitalized active patients aged 20 to 70 years with five-factor score 1 or 2, eosinophil counts 10,034 ± 6641/μL and vasculitis scores 27 ± 6. Overt myocarditis was identified in 10 cases, at disease onset in 6 and relapse in 4, with endomyocarditis in 4 and myopericarditis in 4. Five seronegative and one seropositive patient received rituximab with an induction regimen 375 mg/m2 weekly × 4 for refractory or relapse disease, and the same regimen for annual maintenance therapy. All cases had lower eosinophil counts, improved cardiac dysfunction and clinical remission with a relapse-free follow-up, 48 ± 15 months after the induction treatment. One seronegative endomyocarditis patient had eosinophilia and disease relapse with asthma attack and worsening cardiac insufficiency 24 months after induction, achieving clinical remission under anti-IL-5 therapy. Our findings suggest the suppression of IL-5-mediated eosinophilia as an action mechanism of B-cell-depleting therapy in seronegative EGPA myocarditis.

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Tanaka, Yasuhiro. "Eosinophilic otitis media and eosinophilic granulomatosis with polyangiitis." Journal of Japan Society of Immunology & Allergology in Otolaryngology 36, no. 4 (2018): 271–74. http://dx.doi.org/10.5648/jjiao.36.271.

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Lim, Gareth, Sheryl Lim, Shang-Ian Tee, and Chai Yiing Ling. "A challenging diagnosis of MPO-C-ANCA EGPA." BMJ Case Reports 12, no. 7 (July 9, 2019): e228621. http://dx.doi.org/10.1136/bcr-2018-228621.

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic small-vessel vasculitic disease that can present with positive MPO-P-ANCA (myeloperoxidase–perinuclear–anti-neutrophil cytoplasmic antibody). It is a rare condition that is difficult to diagnose. We present the case of a 64-year-old man with late-onset adult asthma and treated nasopharyngeal carcinoma who initially presented to us with proximal myopathy. Thereafter, he developed a constellation of fleeting symptoms which included rhinosinusitis, mononeuritis multiplex, skin vasculitis and arthritis. Blood investigations showed that he had eosinophilia, and skin biopsy demonstrated dermal vasculitis with eosinophils. He was found to be MPO-C-ANCA positive, and although initially thought to have granulomatosis with polyangiitis, the diagnosis was later revised to EGPA. This case highlights the diagnostic challenges with atypical presentations of EGPA and also presents a rare case of positive MPO-C-ANCA that has never been described in EGPA before.

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Aravinthan, Mythily, Inoshi Atukorala, and Chandu De Silva. "A diagnostic conundrum; Kimura’s disease mimicking eosinophilic granulomatosis with polyangiitis." SAGE Open Medical Case Reports 10 (January 2022): 2050313X2110705. http://dx.doi.org/10.1177/2050313x211070522.

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Kimura’s Disease (KD) is a benign, relapsing chronic inflammatory condition often seen in young Asian males, manifesting as recurrent swellings in skin and subcutaneous tissues especially in the head and neck region. Eosinophilic granulomatosis with polyangitis (EGPA) is a debilitating multisystem vasculitic condition which causes high morbidity due to cavitating lung lesions, neuropathy and renal impairment. Eosinophilia is common to both conditions. We, herein present a young Asian male with KD who presented with isolated recurrent nasopharyngeal mucosal swelling with eosinophilia. Biopsy of the lesion showed necrotizing vasculitis and eosinophilic granulomata mimicking EGPA. Nevertheless, he did not have any other characteristic systemic features of EGPA. Targeted evaluation through multidisciplinary approach helped secure the diagnosis of KD. KD has a wide range of presentation. The three cardinal histopathologic features in KD are eosinophilic inflammation, vascular proliferation and stromal fibrosis. However, vasculitis, granulomata and variable degree of necrosis can be present as in this case. He responded well to a course of steroids followed by Mycophenolate Mofetil. This minimized iatrogenic morbidity to the patient resulting from use of highly toxic immunomodulators which are not necessary in KD unlike in EGPA.

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Uematsu, H., S. Takata, K. Sueishi, and H. Inoue. "Polyangiitis overlap syndrome of granulomatosis with polyangiitis (Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)." Case Reports 2014, feb27 1 (February 27, 2014): bcr2013010195. http://dx.doi.org/10.1136/bcr-2013-010195.

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Alam, Vardah, and Alexandra M. Nanzer. "Eosinophilic granulomatosis with polyangiitis: case report and literature review." Breathe 18, no. 4 (December 2022): 220170. http://dx.doi.org/10.1183/20734735.0170-2022.

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Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg–Strauss syndrome, is a multisystem disorder characterised by asthma, blood and tissue eosinophilia and small-vessel vasculitis. Eosinophilic tissue infiltration and extravascular granuloma formation can lead to damage in any organ, but it is classically seen to cause pulmonary infiltrates, sino-nasal disease, peripheral neuropathy, renal and cardiac involvement, and rashes.EGPA is part of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes, with the antibody being detected in ∼30–40% of cases and mostly against myeloperoxidase. Two genetically and clinically distinct phenotypes, defined by the presence or absence of ANCA have been identified. Treatment for EGPA focuses on inducing and maintaining disease remission. To date, oral corticosteroids remain first-line agents whilst second-line treatments include immunosuppressants such as cyclophosphamide, azathioprine, methotrexate, rituximab and mycophenolate mofetil. However, long-term steroid usage results in multiple and well-known adverse health effects and new insights into the pathophysiology of EGPA have allowed for the development of targeted biologic therapies, like the anti-eosinophilic, anti-interleukin-5 monoclonal antibodies.

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Nakamaru, Yuji. "Eosinophilic granulomatosis with polyangiitis in otolaryngology." Journal of Japan Society of Immunology & Allergology in Otolaryngology 33, no. 3 (2015): 205–7. http://dx.doi.org/10.5648/jjiao.33.205.

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Nakamaru, Yuji. "Diagnosis of Eosinophilic Granulomatosis with Polyangiitis." Practica Oto-Rhino-Laryngologica 115, no. 9 (2022): 818–19. http://dx.doi.org/10.5631/jibirin.115.818.

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Laorden, Daniel, David Romero, and Javier Domínguez-Ortega. "Benralizumab in eosinophilic granulomatosis with polyangiitis." Medicina Clínica (English Edition) 158, no. 9 (May 2022): 441–42. http://dx.doi.org/10.1016/j.medcle.2021.07.018.

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MURAG, SOUMYA, JASON MELEHANI, DARIUS FILSOOF, KARI NADEAU, and R. SHARON CHINTHRAJAH. "DUPILUMAB UNMASKS EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS." Chest 160, no. 4 (October 2021): A8—A9. http://dx.doi.org/10.1016/j.chest.2021.07.065.

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Furuta, Shunsuke, Taro Iwamoto, and Hiroshi Nakajima. "Update on eosinophilic granulomatosis with polyangiitis." Allergology International 68, no. 4 (October 2019): 430–36. http://dx.doi.org/10.1016/j.alit.2019.06.004.

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Nguyen, Yann, and Loïc Guillevin. "Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss)." Seminars in Respiratory and Critical Care Medicine 39, no. 04 (August 2018): 471–81. http://dx.doi.org/10.1055/s-0038-1669454.

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AbstractEosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg–Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five-Factor Score (FFS). One-third of the patients have anti-myeloperoxidase antineutrophil cytoplasm antibodies, and their presence seems to differentiate between two phenotypes, with different clinical characteristics and prognoses. Overall survival has improved markedly since the use of glucocorticoids and immunosuppressants, but relapse rates remain high. All patients require glucocorticoids, and for those with severe/refractory disease and FFS-defined poor prognoses, immunosuppressants should be used (cyclophosphamide for induction and azathioprine for maintenance therapy). Recent advances in EGPA management, including several novel immunomodulatory drugs and targeted biotherapies, were or are being evaluated to improve EGPA patients' prognoses.

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Parperis, Konstantinos, Ray Stanford, Firoozeh Isfahani, Ahmad Chebbo, and Sheetal Chhaya. "Eosinophilic Granulomatosis With Polyangiitis Sine Asthma." American Journal of the Medical Sciences 352, no. 2 (August 2016): e3. http://dx.doi.org/10.1016/j.amjms.2015.11.013.

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Mahr, Alfred, Frank Moosig, Thomas Neumann, Wojciech Szczeklik, Camille Taillé, Augusto Vaglio, and Jochen Zwerina. "Eosinophilic granulomatosis with polyangiitis (Churg–Strauss)." Current Opinion in Rheumatology 26, no. 1 (January 2014): 16–23. http://dx.doi.org/10.1097/bor.0000000000000015.

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48

Guillevin, Loïc. "Mepolizumab for eosinophilic granulomatosis with polyangiitis." Nature Reviews Rheumatology 13, no. 9 (August 3, 2017): 518–19. http://dx.doi.org/10.1038/nrrheum.2017.117.

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Khan, Rizwan, Vickram Tejwani, and Sumita Khatri. "ANCA-Negative Eosinophilic Granulomatosis With Polyangiitis." Chest 150, no. 4 (October 2016): 17A. http://dx.doi.org/10.1016/j.chest.2016.08.020.

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Alba, Marco, J. Jennette, and Ronald Falk. "Pathogenesis of ANCA-Associated Pulmonary Vasculitis." Seminars in Respiratory and Critical Care Medicine 39, no. 04 (August 2018): 413–24. http://dx.doi.org/10.1055/s-0038-1673386.

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AbstractAntineutrophil cytoplasmic antibodies (ANCAs) are autoantibodies specific for antigens located in the cytoplasmic granules of neutrophils and lysosomes of monocytes. ANCAs are associated with a spectrum of necrotizing vasculitis that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Pulmonary vasculitis and related extravascular inflammation and fibrosis are frequent components of ANCA vasculitis. In this review, we detail the factors that have been associated with the origin of the ANCA autoimmune response and summarize the most relevant clinical observations, in vitro evidence, and animal studies strongly indicating the pathogenic potential of ANCA. In addition, we describe the putative sequence of pathogenic mechanisms driven by ANCA-induced activation of neutrophils that result in small vessel necrotizing vasculitis and extravascular granulomatous necrotizing inflammation.

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