2,934 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis


Central retinal artery occlusion in eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome): the first case report in South Korea.

Int J Ophthalmol 2021 18;14(6):948-951. Epub 2021 Jun 18.

Department of Laboratory Medicine, Pusan National University School of Medicine, Biomedical Research Institute, Pusan National University Hospital, Busan 49241, Republic of Korea.

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Prevasculitic Eosinophilic Granulomatosis With Polyangiitis.

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

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COVID-19 and eosinophilic granulomatosis with polyangiitis or COVID-19 mimicking eosinophilic granulomatosis with polyangiitis?

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

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Eosinophilic granulomatosis with polyangiitis: Cutaneous clinical and histopathologic differential diagnosis.

J Cutan Pathol 2021 May 21. Epub 2021 May 21.

Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, but severe systemic vasculitis that can affect skin and other organ systems. Diagnostic criteria have evolved, and many attempts have been made to classify the vasculitides based on clinical and/or histopathologic features, with an aim to develop standardized criteria. According to the EGPA Consensus Task Force recommendations, EGPA is a syndrome of asthma, eosinophilia, pulmonary infiltrates, and extrapulmonary vasculitis (such as cutaneous involvement with purpura). Read More

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ANCA-Negative Churg-Strauss Syndrome Presenting as Bilateral Central Retinal Artery Occlusion: A Case Report

Turk J Ophthalmol 2021 04;51(2):127-130

Birjand University of Medical Sciences Valiasr Hospital, Department of Internal Medicine, Birjand, Iran

A 42-year-old man with undiagnosed Churg-Strauss syndrome (CSS) developed bilateral central retinal artery occlusion (CRAO). His medical history included bronchial asthma and irregular prednisolone usage but no atherosclerotic risk factors. At presentation, visual acuity (VA) was hand motion in the right eye and counting fingers in left eye. Read More

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Clinical characteristics and prognostic factors in an Argentinian cohort with ANCA-associated vasculitis.

Medicina (B Aires) 2021 ;81(2):198-207

Servicio de Inmunología, Instituto de Investigaciones Médicas Alfredo Lanari, Universidad de Buenos Aires, Argentina.

ANCA-associated vasculitis is a heterogeneous group of rare autoimmune conditions of unknown cause. Clinical characteristics and prognostic factors were analyzed in 47 patients: 20 (42.5%) with granulomatosis with polyangiitis, 17 (36. Read More

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Clinical Features of Patients with Active Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab.

Int Arch Allergy Immunol 2021 Apr 22:1-13. Epub 2021 Apr 22.

Department of Pulmonology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

Background: In some patients with eosinophilic granulomatosis with polyangiitis (EGPA), remission cannot be induced, despite treatment with corticosteroids and immunosuppressants. We evaluated the clinical features of patients with EGPA in whom mepolizumab was effective.

Methods: There were 59 EGPA patients treated at Hiratsuka City Hospital, Japan, between April 2018 and September 2020, and 30 of them received mepolizumab. Read More

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Vasculitis therapy refines vasculitis mechanistic classification.

Autoimmun Rev 2021 Jun 16;20(6):102829. Epub 2021 Apr 16.

Department of Biomedicine, Aarhus University, Aarhus, Denmark; Department of Rheumatology, Aarhus University Hospital, Aarhus, Denmark; Department of Rheumatology, Silkeborg Regional Hospital, Silkeborg, Denmark. Electronic address:

The primary vasculitides constitute a heterogeneous group of immune mediated diseases of incompletely understood pathogenesis currently classified by the size of blood vessels affected (Chapel Hill classification). In recent years, several drugs with well-characterized immunological targets have been tested in clinical trials in large vessel vasculitis and small vessel vasculitis. Such trials provide "reverse translational" or bedside to bench information about underlying pathogenic mechanisms. Read More

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Acute heart failure: on the track of a rare disease.

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

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Churg-Strauss Syndrome Along with Cardiac Complications.

Int J Appl Basic Med Res 2021 Jan-Mar;11(1):50-52. Epub 2021 Jan 26.

Health Care Center Sarajevo, Sarajevo, Bosnia and Herzegovina.

Churg-Strauss syndrome (CCS) or eosinophilic granulomatosis with polyangiitis is a rare condition, which is a challenge for both diagnosis and treatment in clinical work. Occurrences of cardiac complications represent a negative predictor of treatment outcome for these patients. The aim of this article is to present the diagnostic and therapeutic modality of a 33-year-old male diagnosed with? CCS. Read More

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January 2021

Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China.

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

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Anti-neutrophil cytoplasmic antibodies-negative Churg-Strauss syndrome presenting as granuloma annulare-like lesions: An unusual cutaneous presentation and a diagnostic pitfall.

Indian J Dermatol Venereol Leprol 2021 Mar-Apr;87(2):259-262

Department of Dermatology, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, P.R. China.

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September 2019

Eosinophil ETosis-mediated release of galectin-10 in eosinophilic granulomatosis with polyangiitis.

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

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Occupational exposures and smoking in eosinophilic granulomatosis with Polyangiitis (Churg-strauss syndrome).

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

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Effectiveness and safety of mepolizumab in combination with corticosteroids in patients with eosinophilic granulomatosis with polyangiitis.

Arthritis Res Ther 2021 03 16;23(1):86. Epub 2021 Mar 16.

The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Kitakyushu, 807-8555, Japan.

Background: Mepolizumab (MPZ), an anti-interleukin-5 antibody, is effective for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA). However, its effectiveness has not been adequately evaluated in real-world clinical practice. In this study, we assessed the effectiveness and safety of MPZ (300 mg) for relapsing/refractory EGPA resistant to corticosteroids (CS) for 1 year in real-world settings. Read More

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Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology.

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

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February 2021

[Cortisone-free rheumatology-Vasculitides].

Z Rheumatol 2021 May 11;80(4):314-321. Epub 2021 Mar 11.

Rheumazentrum Schleswig-Holstein Mitte, Kuhberg 5a-7, 24534, Neumünster, Deutschland.

Glucocorticoids (GC) still represent an essential pillar of treatment in the phase of remission induction of vasculitides, which are often organ or life-threatening; however, they entail a significant potential for side effects. In the phase of remission maintenance prednisolone should be reduced to 7.5 mg/day or less. Read More

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Serum chitinase-3-like 1 protein is a useful biomarker to assess disease activity in ANCA-associated vasculitis: an observational study.

Arthritis Res Ther 2021 03 8;23(1):77. Epub 2021 Mar 8.

Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Republic of Korea.

Background: To investigate whether serum chitinase-3-like 1 protein (YKL-40) is associated with disease activity in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Methods: ELISA was performed in serum samples from AAV patients who were enrolled in our prospective observational cohort to estimate levels of YKL-40. Birmingham vasculitis activity score (BVAS) (version 3), five factor score (FFS), and short form-36 (SF-36), as well as clinical and laboratory data were collected. Read More

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Loeffler's endocarditis in a patient with a new diagnosed Churg-Strauss syndrome (CSS): A case report.

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

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January 2021

Multiple strokes and cerebral microangiopathy in a patient with Churg-Strauss syndrome.

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

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January 2021

Three concurrent morphological presentations of eosinophilic granulomatosis with polyangiitis.

Int J Dermatol 2021 Jun 20;60(6):759-760. Epub 2021 Feb 20.

Division of Dermatology, Department of Medicine, National University Healthcare System, Singapore.

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A proposed role of neutrophil extracellular traps and their interplay with fibroblasts in ANCA-associated vasculitis lung fibrosis.

Autoimmun Rev 2021 Apr 18;20(4):102781. Epub 2021 Feb 18.

Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico. Electronic address:

ANCA-associated vasculitides (AAV) comprise three diseases: granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. They are characterised by small vessel inflammation and have a broad range of clinical manifestations and multiorgan involvement which endanger the patient's life. An increasingly recognised complication of AAV, especially in MPA is lung fibrosis, for which no clearcut therapy in this context is available. Read More

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The association between ear involvement and clinical features and prognosis in ANCA-associated vasculitis.

Auris Nasus Larynx 2021 Oct 13;48(5):885-889. Epub 2021 Feb 13.

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

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October 2021

Case Report: A 64-Year-Old Man with 10-Year History of Eosinophilic Granulomatosis with Polyangiitis with Bronchiectasis and Severe Klebsiella pneumonia.

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

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February 2021

Otologic Manifestations of Eosinophilic Granulomatosis With Polyangiitis: A Systematic Review.

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

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Polyangiitis overlap syndrome: a novel presentation of microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.

BMJ Case Rep 2021 Feb 1;14(2). Epub 2021 Feb 1.

Internal Medicine, Highland Hospital, Oakland, California, USA.

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. Read More

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February 2021

Low-dose corticosteroid therapy improves refractory coronary vasospasm accompanied by eosinophilic granulomatosis with polyangiitis.

J Cardiol Cases 2021 Feb 10;23(2):69-72. Epub 2020 Oct 10.

Department of Internal Medicine, Saiseikai Hyogoken Hospital, Kobe, Japan.

A 57-year-old man was admitted to our hospital due to repeated chest pain. Coronary spastic angina was diagnosed by emergent coronary angiography. His chest attack was not suppressed with vasodilator therapy; however, it finally improved after administration of 20 mg prednisolone. Read More

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February 2021

The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy.

Intern Med 2021 Jun 1;60(12):1963-1969. Epub 2021 Feb 1.

Department of Nephrology, National Center for Global Health and Medicine, Japan.

The first case of eosinophilic granulomatosis with polyangiitis (EGPA) simultaneously demonstrating various clinical manifestations, including retroperitoneal fibrosis (RPF) causing hydronephrosis and membranous nephropathy (MN) leading to nephrotic syndrome, is presented. There have been no previous case reports demonstrating the simultaneous onset of these three disease categories with significant complex pathologies. This case was successfully managed by providing adequate combination therapies according to each disease category, leading to complete remission (CR) of all three diseases. Read More

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