Article
Immunology
Beatrice Maranini, Ippolito Guzzinati, Gian Luca Casoni, Maria Ballotta, Andrea Lo Monaco, Marcello Govoni
Summary: This article presents the first detailed description of a case of MLS associated with EGPA that was completely resolved through FOB. Identifying MLS in EGPA is essential as prognosis, longitudinal management, and treatment options may differ from other pulmonary involvement in AAV patients.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Antonios G. A. Kolios, Andreas Lutterotti, Zsolt Kulcsar, Tobias Renner, Alain Rudiger, Jakob Nilsson
Summary: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a small-vessels vasculitis associated with ANCA, characterized by hypereosinophilia and eosinophilic asthma. In cases of life-threatening organ involvement, particularly cardiac and central nervous system (CNS), immediate immunosuppression is required. Antibiotic therapy combined with Benralizumab can be a potential therapeutic option for active EGPA with eosinophil-mediated pathologies in severely ill patients.
CLINICAL IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
Alexandra Villa-Forte
Summary: This review provides an overview of the epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of eosinophilic granulomatosis with polyangiitis (EGPA). EGPA is a rare small to medium vessel necrotizing vasculitis that is often misdiagnosed due to its evolving nature. The unique features of EGPA include asthma and eosinophilia. The disease commonly presents with polyneuropathies and heart involvement, which are often severe and require aggressive immunosuppressive therapy. The treatment approach depends on the severity of the disease, with more severe cases requiring aggressive therapies such as cyclophosphamide. Asthma management often requires prolonged treatment with corticosteroids due to the high rate of disease relapses. Improved understanding of the heterogeneity of the disease is necessary for the development of better therapies.
POSTGRADUATE MEDICINE
(2023)
Article
Rheumatology
Silvia Sartorelli, Guillaume Chassagnon, Pascal Cohen, Bertrand Dunogue, Xavier Puechal, Alexis Regent, Luc Mouthon, Loic Guillevin, Benjamin Terrier
Summary: Specific cardiomyopathy is frequent in EGPA, especially in ANCA-negative patients with high eosinophil counts. Long-term outcome was better than previously reported. Abnormal ECG and LGE on CMRI were associated with the occurrence of MACEs.
Article
Immunology
Rongli Li, Yingying Chen, Shangzhu Zhang, Linyi Peng, Jiaxin Zhou, Yunyun Fei, Wen Zhang, Yan Zhao, Xiaofeng Zeng
Summary: This study aimed to investigate the clinical characteristics, potential risk factors, and long-term outcomes in EGPA patients with gastrointestinal (GI) involvement. The results showed that EGPA patients with GI involvement had higher levels of hs-CRP, higher grades of BVAS and FFS scores compared to those without GI involvement. During the follow-up period, EGPA patients with GI involvement had lower cumulative survival rate, lower long-term remission rate, and higher cumulative relapse rate.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Immunology
J. P. E. White, S. Dubey
Summary: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease characterized by late-onset asthma, eosinophilia, and vasculitis. It is different from other anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and has a unique pathophysiology. There is currently no standard therapy for EGPA, and further research is needed to understand its complex pathogenesis and develop more effective treatment options.
AUTOIMMUNITY REVIEWS
(2023)
Article
Rheumatology
Matthias Papo, Renato A. Sinico, Vitor Teixeira, Nils Venhoff, Maria-Letizia Urban, Michele Iudici, Juliane Mahrhold, Francesco Locatelli, Giulia Cassone, Franco Schiavon, Benjamin Seeliger, Thomas Neumann, Claus Kroegel, Matthieu Groh, Chiara Marvisi, Maxime Samson, Thomas Barba, David Jayne, Arianna Troilo, Jens Thiel, Bernhard Hellmich, Sara Monti, Carlomaurizio Montecucco, Carlo Salvarani, Jean-Emmanuel Kahn, Bernard Bonnotte, Cecile-Audrey Durel, Xavier Puechal, Luc Mouthon, Loic Guillevin, Giacomo Emmi, Augusto Vaglio, Benjamin Terrier
Summary: PR3-ANCA EGPA patients differ from MPO-ANCA and ANCA-negative patients in clinical presentation, but share similarities with granulomatosis with polyangiitis, suggesting it may be a distinct form of PR3-ANCA-associated vasculitis.
Review
Medicine, General & Internal
Filippo Fagni, Federica Bello, Giacomo Emmi
Summary: EGPA is a rare multisystemic disease characterized by vessel inflammation and eosinophilic proliferation. It is divided into different phenotypes based on ANCA status, with diagnosis and treatment implications.
FRONTIERS IN MEDICINE
(2021)
Review
Immunology
Mitsuhiro Akiyama, Yuko Kaneko, Tsutomu Takeuchi
Summary: Rituximab was found effective in inducing and sustaining remission, reducing glucocorticoid dose, and potentially providing greater benefit in ANCA-positive patients with EGPA. Scheduled maintenance treatment with rituximab significantly decreased relapse rates compared to on-demand administration, with no new safety concerns reported.
AUTOIMMUNITY REVIEWS
(2021)
Article
Immunology
Santi Nolasco, Andrea Portacci, Raffaele Campisi, Enrico Buonamico, Corrado Pelaia, Alida Benfante, Massimo Triggiani, Giuseppe Spadaro, Maria Filomena Caiaffa, Giulia Scioscia, Aikaterini Detoraki, Giuseppe Valenti, Francesco Papia, Alessandra Tomasello, Nunzio Crimi, Nicola Scichilone, Girolamo Pelaia, Giovanna Elisiana Carpagnano, Claudia Crimi
Summary: This study suggests that anti-IL-5/Rα biologics are effective and safe as long-term add-on treatments for patients with EGPA.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Medicine, General & Internal
Roberto Rios-Garces, Sergio Prieto-Gonzalez, Jose Hernandez-Rodriguez, Ebymar Arismendi, Isam Alobid, Alessandra E. Penatti, Maria C. Cid, Georgina Espigol-Frigole
Summary: In patients with EGPA, treatment with mepolizumab can improve asthma control and reduce flare frequency, but its effectiveness in improving ENT symptoms is limited.
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2022)
Article
Allergy
Vamsi P. Guntur, Laurie A. Manka, Joshua L. Denson, Ryan M. Dunn, Yeshai T. Dollin, Mary Gill, Christena Kolakowski, Matthew J. Strand, Michael E. Wechsler
Summary: The study demonstrated that benralizumab was well tolerated in patients with EGPA, facilitating reduction in oral corticosteroid dose and decreasing exacerbations. Larger controlled trials are needed to further evaluate the potential role of benralizumab in treating EGPA.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2021)
Article
Rheumatology
Alessandra Milanesi, Paolo Delvino, Silvana Quaglini, Carlomaurizio Montecucco, Sara Monti
Summary: This study retrospectively collected data from 57 patients with eosinophilic granulomatosis with polyangiitis to compare the effectiveness, safety, and steroid-sparing effect of AZA and MTX as induction and maintenance therapy. The results showed that MTX1 had a higher and earlier remission rate, while MTX2 had a better steroid-sparing effect.
Review
Rheumatology
Bahar Ozdemir, Abdulsamet Erden, Serdar Can Guven, Berkan Armagan, Hakan Apaydin, Ozlem Karakas, Ahmet Gokhan Akdag, Ihsan Ates, Orhan Kucuksahin, Ahmet Omma
Summary: COVID-19 and eosinophilic granulomatosis with polyangiitis (EGPA) can present similarly in clinical and imaging findings, making it a diagnostic challenge to differentiate between the two during the current pandemic, especially when other EGPA symptoms are overlooked. This study reviewed cases involving EGPA patients with COVID-19, as well as patients initially suspected to have COVID-19 but eventually diagnosed with EGPA, providing insights into navigating the diagnostic challenge presented by EGPA/COVID-19 during the pandemic.
RHEUMATOLOGY INTERNATIONAL
(2021)
Article
Medicine, General & Internal
Yongzhen Chen, Qiuxia Wan, Bo Liu
Summary: A 29-year-old male patient was admitted to the hospital with fever and rash on both lower extremities, and symptoms of muscle pain, nausea, anorexia, abdominal pain, and diarrhea. After diagnosis, the patient was confirmed to have systemic EGPA and was treated with medication, resulting in the improvement of most symptoms but no improvement in limb weakness.
