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Cureus 2021 Apr 23;13(4):e14649. Epub 2021 Apr 23.

Critical Care Medicine, University of Pittsburgh Medical Center Mercy, Pittsburgh, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an exceptionally rare systemic necrotizing vasculitis. The disease is clinically characterized by asthma with concomitant blood and tissue eosinophilia, often progressing to eosinophilic vasculitis. From the onset of asthma, there is usually a three to nine year delay of EGPA diagnosis. Read More

Rheumatol Int 2021 May 25. Epub 2021 May 25.

Division of Rheumatology, Department of Internal Medicine, Ankara City Hospital, Ankara, 06100, Turkey.

Coronavirus disease 2019 (COVID-19) and eosinophilic granulomatosis with polyangiitis (EGPA) share similarities in clinical, imaging findings and may present with respiratory distress. Differentiating a new-onset EGPA from COVID-19 during the current pandemic is a diagnostic challenge, particularly if other EGPA symptoms are overlooked. Here in this study we reviewed the literature regarding EGPA patients with COVID-19 and patients who diagnosed with EGPA or suffered an EGPA flare mimicking COVID-19. Read More

BMJ Case Rep 2021 Apr 14;14(4). Epub 2021 Apr 14.

Cardiology Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.

Eosinophilic granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis with cardiac involvement in more than 60% of cases. Authors describe the case of a 48-year-old woman who presented with progressively worsening asthenia, dyspnoea and macular, non-painful, non-itchy cutaneous lesions. She had signs of congestion on clinical examination and a history of asthma and nasal polyps. Read More

Orphanet J Rare Dis 2021 Mar 31;16(1):152. Epub 2021 Mar 31.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, the Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, 100730, China.

Background: Central nervous system (CNS) involvement is extremely rare in eosinophilic granulomatosis with polyangiitis (EGPA), but is associated with a poor prognosis in the five-factor score. This study aims to elucidate the clinical features and independently associated factors of EGPA with CNS involvement.

Results: CNS involvement was observed in 17. Read More

Arthritis Rheumatol 2021 Mar 22. Epub 2021 Mar 22.

National Hospital Organization Sagamihara National Hospital, Clinical Research Center, Kanagawa, Japan.

Objective: Eosinophils are tissue-dwelling immune cells.　Accumulating evidence indicates that a type of cell death termed ETosis is an important cell fate involved in the pathophysiology of inflammatory diseases. Although the critical role of eosinophils, in eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is well established, the presence of eosinophil ETosis (EETosis) is poorly understood. Read More

Arthritis Rheumatol 2021 Mar 22. Epub 2021 Mar 22.

Department of Medicine and Surgery, University of Parma, Parma.

Objective: Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Environmental agents and occupational exposures may confer susceptibility to EGPA, but data are scarce. We investigated the association between occupational exposures (e. Read More

Front Med (Lausanne) 2021 24;8:627776. Epub 2021 Feb 24.

Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy.

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare multisystemic disease classified both amongst hypereosinophilic disorders and ANCA-associated vasculitis. Vessel inflammation and eosinophilic proliferation are the hallmarks of the disease and main effectors of organ damage. Two distinct disease phenotypes have classically been described according to ANCA-status: the ANCA-negative subset with eosinophil-driven manifestation and the ANCA-positive one with vasculitic manifestations. Read More

Caspian J Intern Med 2021 ;12(1):107-110

Department of Cardiology, Cheng Hsin General Hospital, Taipei, Taiwan.

Background: Loeffler's endocarditis is a rare disease, caused by endocardial involvement of esosinophils, which damages the heart and leads to endomyocardial fibrosis with consequent restrictive cardiomyopathy, mural thrombi or valvular dysfunction. The association between Loeffler's endocarditis and Churg-Strauss syndrome (CSS) was also reported. Abnormal elevation of peripheral eosinophil counts in a heart failure patient is a hint of disease. Read More

Med Pharm Rep 2021 Jan 29;94(1):121-124. Epub 2021 Jan 29.

Department of Neurology Science, University of Medicine and Pharmacy Cluj-Napoca, Romania.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disease that mainly affects the lungs and skin. It is considered to be a small and medium-vessel vasculitis. Although neurologic manifestations of EGPA are reported, usually consisting of peripheral neuropathy, central nervous system manifestations are quite rare, those described being cerebral infarctions or hemorrhages. Read More

Auris Nasus Larynx 2021 Feb 12. Epub 2021 Feb 12.

Department of Otolaryngology, Head and Neck Surgery, Ehime University Graduate School of Medicine, Toon, Japan.

Objective: The concept of otitis media with ANCA-associated vasculitis (OMAAV) was recently proposed by the study group of the Japan Otological Society. However, little is known about the effect of ear involvement on the clinical features and prognosis of AAV. We investigate this issue in this study. Read More

Am J Case Rep 2021 Feb 14;22:e929224. Epub 2021 Feb 14.

Laboratory for the Study of Advanced Simulation Technologies, V. A. Negovsky Research Institute of General Reanimatology, Federal Research and Clinical Center of Intensive Care Medicine and Rehabilitology, Moscow, Russian Federation.

BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and severe progressive disease with multiple clinical manifestations and organ damage. Usually, it requires long-term monitoring of the state of many organs due to the gradual character of its manifestations. CASE REPORT We report a case of a long-term follow-up of a patient with eosinophilic granulomatosis with polyangiitis with emphasis on specific clinical features in this patient. Read More

Otol Neurotol 2021 04;42(4):e380-e387

Hackensack University Medical Center, Hackensack, New Jersey.

Objective: To perform a systematic review of the diagnosis, treatment, and management of patients with otologic manifestations of eosinophilic granulomatosis with polyangiitis.

Databases Reviewed: PubMed, Embase, Cochrane.

Methods: A systematic search for relevant published literature in PubMed, Cochrane Library, and EMBASE databases was done. Read More

Clin Case Rep 2021 Jan 11;9(1):547-550. Epub 2020 Dec 11.

Department of Pediatrics Japan Community Healthcare Organization Kyushu Hospital Kitakyushu Japan.

A 29-year-old man after the Fontan operation had a catastrophic intracranial hemorrhage associated with eosinophilic granulomatous polyangiitis. Despite combination therapy with cyclophosphamide and methylprednisolone, he was dead at 6 months after the onset. The clinical course was worse owing to underlying coagulopathy and endothelial dysfunction associated with congenital heart disease. Read More

Arthritis Res Ther 2021 01 14;23(1):28. Epub 2021 Jan 14.

Department of Nephrology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.

Objective: To assess through systematic review and meta-analysis whether plasma exchange (PE) is associated with prognosis in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients.

Methods: A systematic search of PubMed, MEDLINE, Embase, and CENTRAL databases from inception to 17 June 2020 was conducted. Ongoing or unpublished trials were also searched in ClinicalTrials. Read More

Eur Heart J Case Rep 2020 Dec 5;4(6):1-5. Epub 2020 Nov 5.

Department of Cardiovascular Medicine, Lady Davis Carmel Medical Center, 7 Michal Street, Haifa 34362, Israel.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare multisystem disease characterized by asthma, rhinosinusitis, and eosinophilia. Cardiac involvement, present in half the patients, may be life threatening.

Case Summary: A young woman with long-standing asthma and nasal polyposis was admitted with new-onset dyspnoea, sinus tachycardia, and eosinophilia. Read More

Eur J Intern Med 2021 Mar 23;85:68-79. Epub 2020 Dec 23.

Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis. Cardiac specific involvement (CSI) is caused by coronary artery vasculitis, but also by myocardial eosinophilic infiltration. To date, the prevalence of CSI associated with EGPA is unresolved. Read More

Autoimmun Rev 2021 Feb 17;20(2):102737. Epub 2020 Dec 17.

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Japan.

Objective: We investigated the effectiveness of rituximab (an anti-CD20 monoclonal antibody) in patients with eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: We performed a systematic literature review from the inception dates until July 20, 2020 for articles reporting rituximab administration to treat EGPA.

Results: We identified a total of 171 patients; most of the patients had refractory or relapsing disease, whereas 14 patients were newly diagnosed with EGPA. Read More

Tidsskr Nor Laegeforen 2020 12 14;140(18). Epub 2020 Dec 14.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterised by chronic rhinosinusitis, asthma, and pronounced peripheral blood eosinophilia. The most commonly involved organ is the lung. However, EGPA can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems. Read More

J Dermatol 2021 Apr 10;48(4):529-532. Epub 2020 Dec 10.

Division of Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

Treatment of eosinophilic granulomatosis with polyangiitis (EGPA) remains a challenge because currently available therapies, corticosteroids and immunomodulators, do not always control symptoms and are often associated with significant morbidity and relapse. Mepolizumab has been demonstrated to be an effective add-on therapy with steroid-sparing effect in cases of relapsing or refractory EGPA. Intravenous immunoglobulin (IVIG) therapy is effective against mononeuritis multiplex or heart failure in patients with EGPA who do not respond to corticosteroid-cyclophosphamide treatment. Read More

Int J Cardiovasc Imaging 2021 Apr 19;37(4):1371-1381. Epub 2020 Nov 19.

Manchester University NHS Foundation Trust, Wythenshawe Hospital, Southmoor Road, Wythenshawe, Manchester, M23 9LT, UK.

Patients with eosinophilic granulomatosis with polyangiitis (EGPA) most commonly die from cardiac causes, however, cardiac involvement remains poorly characterised and the relationship between cardiac and pulmonary disease is not known. This study aimed to characterise myocardial and pulmonary manifestations of EGPA, and their relationship. Prospective comprehensive cardiopulmonary investigation, including a novel combined cardiopulmonary magnetic resonance imaging (MRI) technology, was performed in 13 patients with stable EGPA. Read More

Clin Exp Rheumatol 2021 Mar-Apr;39 Suppl 129(2):114-118. Epub 2020 Nov 10.

Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, ON, Canada.

Objectives: Only a few small case series, case reports, and one small clinical trial suggested some benefit of leflunomide (LEF) in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and other vasculitides. We analysed the clinical efficacy and tolerability of LEF in a large cohort of patients with various vasculitides.

Methods: This was a retrospective analysis of patients who received LEF for treatment of their vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Study and in 3 additional centres from the Canadian vasculitis research network (CanVasc). Read More

Clin Imaging 2021 Feb 31;70:124-135. Epub 2020 Oct 31.

University of Toronto - Sunnybrook Health Sciences Centre, Department of Medical Imaging, 2075 Bayview Ave, Toronto, ON M4N 3M5, Canada. Electronic address:

Certain entities may simultaneously involve the lungs and the myocardium. Knowing their cardiac and thoracic manifestations enhances the understanding of those conditions and increases awareness and suspicion for possible concurrent cardiothoracic involvement. Entities that can present with pulmonary and myocardial involvement include infiltrative diseases like sarcoidosis and amyloidosis, eosinophil-associated conditions including eosinophilic granulomatosis with polyangiitis (EGPA) and hypereosinophilic syndrome (HES), connective tissue diseases such as systemic sclerosis (SSc) and lupus erythematosus and genetic disorders like Fabry disease (FD). Read More

Rheumatology (Oxford) 2021 01;60(1):1-2

Department of Psychology, Washington State University Vancouver, Vancouver, WA, USA.

Neurology 2021 02 27;96(5):226-229. Epub 2020 Oct 27.

From the Department of Neurology (R.N., H.K., K.O., Y.F., M.I., M.K.), Nagoya University Graduate School of Medicine; Department of Neurology (K.O.), Okazaki City Hospital; and Research Division of Dementia and Neurodegenerative Disease (G.S.), Nagoya University Graduate School of Medicine, Japan.

Clin Rheumatol 2021 May 22;40(5):1949-1957. Epub 2020 Oct 22.

Department of Neuroscience-DNS, Otolaryngology Section, Padova University, Via Giustiniani 2, 35128, Padova, Italy.

Introduction/objectives: The histopathological study of inflammatory cells and their tendency to form aggregates in chronic rhinosinusitis with nasal polyps (CRSwNP) has shown promising results in determining the pathogenesis and predicting clinical outcome. Bilateral nasal polyps also occur in over 70% of patients with eosinophilic granulomatosis with polyangiitis (EGPA). The study aim was to investigate neutrophil infiltrates and eosinophil aggregates in CRSwNP and EGPA tissues of Caucasian patients. Read More

J Allergy Clin Immunol Pract 2021 03 15;9(3):1107-1117.e2. Epub 2020 Oct 15.

Dartmouth-Hitchcock Medical Center, Section of Allergy and Immunology, Lebanon, NH; Geisel School of Medicine at Dartmouth, Departments of Pediatrics, of Medicine, and of Community and Family Medicine, Hanover, NH.

The introduction of specific humanized monoclonal antibodies over the past 20 years has dramatically changed the treatment of allergic diseases. At present, 5 mAbs are licensed for treating moderate to severe allergic and eosinophilic asthma, atopic dermatitis, chronic spontaneous urticaria, chronic sinusitis with nasal polyps, and eosinophilic granulomatosis with polyangiitis. Given the high costs of biologics, understanding their cost-effectiveness is critical. Read More

J Investig Med High Impact Case Rep 2020 Jan-Dec;8:2324709620966855

UCLA-Kern Medical, Bakersfield, CA, USA.

Vasculitis is an inflammatory process involving blood vessels of various sizes, including the small vessels in the kidneys to the large vessels, such as the aorta. This inflammatory condition is usually autoimmune in nature and is associated with involvement of many locations, such as the sinuses, lungs, kidneys, and even the heart. Specifically, eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis that may initially hide as asthma, allergic rhinitis, and/or sinusitis. Read More

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2020 Aug;45(8):916-922

Department of Nephrology, Xiangya Hospital, Central South University, Changsha 410008.

Objectives: To investigate the clinic-pathological characteristics, prognosis and its risk factors for antineutrophil cytoplasimc antibody (ANCA)-associated vasculitis (AAV).

Methods: The basic information and clinic-pathological characteristics of AAV patients, who was diagnosed from January 2010 to January 2018 in Xiangya Hospital, Central South University, were retrospectively collected. The renal survival and patient survival were regular followed up, and their clinical pathological, prognosis data and risk factors for renal were analyzed. Read More

Cureus 2020 Sep 6;12(9):e10279. Epub 2020 Sep 6.

Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune systemic necrotizing vasculitis of blood vessels that often presents with hypereosinophilia. Cardiac involvement in EGPA directly correlates with the mortality of patients with the disease and is a central part of the disease process. The evaluation and treatment of cardiac anomalies are vital in patients with EGPA. Read More

Allergy 2020 12 23;75(12):3087-3099. Epub 2020 Oct 23.

Center for Allergy and Immunology, Shonan Kamakura General Hospital, Kanagawa, Japan.

Eosinophilic airway inflammation is one of the cardinal features of allergic airway diseases such as atopic asthma and allergic rhinitis. These childhood-onset conditions are mediated by allergen and allergen-specific IgE and often accompanied by other allergic diseases including food allergy and eczema. They can develop consecutively in the same patient, which is referred to as an allergic march. Read More