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
Multidisciplinary Sciences
Takumi Tashiro, Urara Fujiwara, Yuichi Kira, Chihiro Karashima, Norihisa Maeda
Summary: A 77-year-old woman with sinusitis developed embolic stroke and splenic infarctions, and was diagnosed with eosinophilic granulomatosis with polyangiitis characterized by eosinophilic infiltration. Cardiac imaging also revealed evidence of endo-myocarditis. The patient received immunosuppressive and anticoagulation therapy, and no recurrence of infarctions was observed.
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
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.
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.
Article
Immunology
Pierre M. Bataille, Cecile-Audrey Durel, Dominique Chauveau, Arnaud Panes, Eric Simon Thervet, Benjamin Terrier
Summary: A study conducted in France revealed that despite advances in therapeutic management, the mortality rates of GPA and MPA patients remain high and stable, highlighting the need for further improvement in management.
JOURNAL OF AUTOIMMUNITY
(2022)
Article
Medicine, General & Internal
Giorgia Carnicelli, Alvise Sernicola, Vito Gomes, Giulia Cundari, Stefania Trasarti, Roberta Priori, Teresa Grieco
Summary: This article reports the clinical features and cutaneous manifestations of an ANCA-positive EGPA patient, emphasizing the importance of dermatologic signs in diagnosis and treatment.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Rheumatology
Maxime Samson, Herve Devilliers, Sara Thietart, Pierre Charles, Christian Pagnoux, Pascal Cohen, Alexandre Karras, Luc Mouthon, Benjamin Terrier, Xavier Puechal, Loic Guillevin
Summary: The study aimed to develop a score for assessing the likelihood of relapse in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Long-term follow-up data from patients with GPA and MPA included in five randomized controlled trials were pooled. Variables associated with relapse were identified and used to build a score, which was validated in an independent cohort.
Review
Rheumatology
Jan Henrik Schirmer, Beatriz Sanchez-Alamo, Bernhard Hellmich, David Jayne, Sara Monti, Raashid Ahmed Luqmani, Gunnar Tomasson
Summary: This systematic literature review provides evidence on the treatment of antineutrophil cytoplasm antibody-associated vasculitis (AAV) and suggests that cyclophosphamide and rituximab have similar efficacy for remission induction, but rituximab is more effective in relapsing disease. Faster tapering glucocorticoid protocols result in similar remission rates but lower rates of serious infections. Avacopan shows potential for rapid tapering and replacing glucocorticoids. Use of rituximab for maintenance of remission is associated with lower relapse rates compared with azathioprine. Prolonged maintenance treatment reduces relapse rates for both azathioprine and rituximab.
Article
Medicine, General & Internal
Tapas Ranjan Behera, Amninder Kaur, Satyajit Acharya, Prabhat Mallick, Alisha Sahu
Summary: This article reports a case of a 23-year-old female patient presenting with symptoms such as dyspnea, hemoptysis, hypoxemia, anemia, and oliguria. After various treatment measures, the patient's symptoms and radiographic findings improved.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Immunology
Roberto Padoan, Mariele Gatto, Anna Ghirardello, Marta Tonello, Chiara Franco, Mara Felicetti, Franco Schiavon, Andrea Doria
Summary: The study found a high prevalence of anti-PTX3 antibodies in AAV patients, especially in those with EGPA. Anti-PTX3 antibodies were associated with specific disease manifestations, suggesting their potential as a novel biomarker for AAV.
JOURNAL OF AUTOIMMUNITY
(2021)
Review
Rheumatology
Julia A. Ford, Yaseen Aleatany, Ora Gewurz-Singer
Summary: This review summarizes the recent advances in the management of eosinophilic granulomatosis with polyangiitis (EGPA), with a focus on the use of biologic therapies. The anti-interleukin (IL)-5 agent mepolizumab has shown efficacy in inducing and maintaining remission, particularly in patients with asthma and allergic manifestations. Rituximab is recommended for remission induction in severe disease, especially in ANCA-positive patients with vasculitic manifestations. However, the evidence for the use of traditional DMARDs and other biologic agents is limited. Advances in biologics have expanded the treatment options for EGPA.
CURRENT OPINION IN RHEUMATOLOGY
(2022)
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)
Article
Rheumatology
Shuang Tang, Xin Li, Kai-Ya Zhao, Qian Zhou, Xiao-Kui Tang
Summary: Microscopic polyangiitis (MPA) with diffuse alveolar hemorrhage (DAH) commonly affects patients over 65 years old, with high rates of pulmonary and renal involvement. The cumulative survival rates at 1, 3, and 5 years were 63.7%, 51.2%, and 47.3% respectively. Age, serum creatinine level, PaO2/FiO2 ratio, and extent of lung involvement were identified as independent prognostic factors for MPA with DAH.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Rheumatology
Federica Bello, Alessandra Bettiol, Elena Silvestri, Irene Mattioli, Maria Letizia Urban, Adalgisa Palermo, Matteo Mazzetti, Danilo Malandrino, Ilenia Calcaterra, Augusto Vaglio, Matteo Nicola Dario Di Minno, Giacomo Emmi, Domenico Prisco
Summary: Ultrasound markers of subclinical atherosclerosis are increased in EGPA patients as compared with controls, independently of traditional cardiovascular risk factors.
