3,124 results match your criteria Churg-Strauss Syndrome


Eosinophilic Granulomatosis with Polyangiitis.

Int J Appl Basic Med Res 2022 Apr-Jun;12(2):148-150. Epub 2022 May 10.

Department of Pharmacology, Dr. DY Patil Medical College, Navi Mumbai, Maharashtra, India.

Eosinophilic granulomatosis with polyangiitis also known as Churg-Strauss syndrome or allergic granulomatosis and angiitis is an extremely rare systemic necrotizing vasculitis that affects small-to-medium-sized vessels. Here, we report a case of a 58-year-old man presenting with painful swelling of bilateral lower limbs with multiple well-defined erosions on lower legs and dorsum of the foot with a few of them showing brownish crusts and with slight watery discharge and a few with raw areas. The patient is a known case of bronchial asthma. Read More

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[Dilated cardiomyopathy in a patient with Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)].

Rev Fac Cien Med Univ Nac Cordoba 2022 06 6;79(2):193-196. Epub 2022 Jun 6.

Sanatorio Allende, Servicio de Cardiología. Córdoba, Argentina..

Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss Syndrome, is a rare pathology that belongs to the group of diseases characterized by necrotizing vasculitis of small and medium-sized systemic blood vessels. Symptomatic cardiovascular involvement occurs in 27% to 47% of Churg-Strauss cases, being one of the most serious manifestations. The diagnosis is usually confirmed with tissue biopsy showing eosinophil infiltration, but in selected cases with the recent inclusion of cardiac MRI, we can dispense with it. Read More

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Sclerokeratitis in Granulomatosis with Polyangiitis.

N Engl J Med 2022 06 4;386(23):2221. Epub 2022 Jun 4.

John A. Moran Eye Center, Salt Lake City, UT

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A Surgical Presentation of Churg-Strauss Syndrome.

Cureus 2022 Apr 21;14(4):e24342. Epub 2022 Apr 21.

Department of Surgery, University Hospital Galway, Galway, IRL.

Eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome (CSS) is a rare, autoimmune vasculitis usually affecting small and medium-sized blood vessels in its later phases. It is a diffuse, systemic, multisystem disease that is reported to present with gastrointestinal manifestations but very rarely as an acute abdomen secondary to eosinophilic peritonitis. A 28-year-old relatively healthy male with a pre-existing diagnosis of inactive pulmonary sarcoidosis presented to the emergency department with an acute abdomen. Read More

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[Steroids-Resistant EGPA-Induced Neuropathy with Acute Disease Progression: How to Use Mepolizumab as Novel Targeted Agent].

Authors:
Naoki Hattori

Brain Nerve 2022 May;74(5):531-536

Toyota Kosei Hospital.

Eosinophilic granulomatosis with polyangiitis (EGPA), an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a systemic vasculitis syndrome involving inflammatory damage of predominantly small vessels. Initial treatment is extremely important because the peripheral nervous system is a major target organ that depends on long-term clinical outcomes. Moreover, detailed neurological observations are necessary during the remission period. Read More

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[Steroids-Resistant EGPA-Induced Neuropathy with Acute Disease Progression: How to Select Remission-Induction/Maintenance Therapy Based on The Characteristics].

Authors:
Yukio Takeshita

Brain Nerve 2022 May;74(5):525-530

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.

Recently, given the availability of mepolizumab as a novel treatment for eosinophilic polyangiitis granulomatosis (EGPA), several studies on remission-induction/maintenance therapies are in progress. However, there is little evidence regarding the treatment of EGPA neuropathy. In this article, we clarify the characteristics of steroid-resistant EGPA neuropathy by presenting actual cases and explaining the selection of remission-induction/maintenance therapies based on the characteristics. Read More

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[ANCA-Associated Vasculitides].

Ther Umsch 2022 Jun;79(5):229-237

Klinik für Rheumatologie, Universitätsspital Basel.

ANCA-Associated Vasculitides The according to their immunoserological markers (anti-neutrophil cytoplasmic antibodies - ANCA) named ANCA-associated vasculitides (AAV) are classified following the Chapel Hill nomenclature (2012). Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are categorized according to clinical, histological, and imaging findings. GPA and EGPA mainly differ from MPA in the presence of granulomatous inflammation within the airways. Read More

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[SUCCESSFUL TREATMENT FOR OTITIS MEDIA AND SINUSITIS ASSOCIATED WITH EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS USING ANTI-IL-5 RECEPTOR MONOCLONAL ANTIBODY BENRALIZUMAB].

Arerugi 2022 ;71(3):242-247

Department of Otolaryngology, Tokyo-kita Medical Center.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of ANCA-related vasculitis. Asthma and sinusitis occur first in the course of EGPA, followed by vasculitis symptoms such as fever, weight loss, and peripheral neuropathy. Otitis media with effusion and sensorineural hearing loss occur occasionally in EGPA patients. Read More

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Added value of Joint ENT-Rheumatology clinic in the management of ANCA-associated vasculitis: One year's experience.

Am J Otolaryngol 2022 Jul-Aug;43(4):103485. Epub 2022 May 5.

ENT Department, Sheffield Teaching Hospitals NHS Trust, Sheffield, UK.

Purpose: ANCA-associated vasculitides (AAV) represent a group of diagnoses, including granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA). Most commonly, they present initially with ENT-associated symptomatology, and therefore they often pose a diagnostic challenge. We aim to present our one-year experience in the joint management of AAV in a multi-disciplinary setting. Read More

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Cerebral Angiography of Eosinophilic Granulomatosis with Polyangiitis.

Neurol India 2022 Mar-Apr;70(2):839-840

Department of Neurology, Ina Central Hospital, 1313-1, Ina, Nagano, Japan.

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Successful benralizumab for eosinophilic myocarditis in eosinophilic granulomatosis with polyangiitis.

Clin Exp Rheumatol 2021 Oct 4;40(4):834-837. Epub 2022 May 4.

Department of Infectious Diseases, Nord Franche-Comté Hospital, Trévenans, France.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterised by many features, including asthma, allergic rhinitis, peripheral and tissue eosinophilia, and vasculitis. Its pathophysiology is still unclear and we suggest that there are different phenotypes of EGPA, which may respond differently to available treatments. Within the most promising targeting biotherapy, benralizumab, an anti-interleukin-5 receptor alpha monoclonal antibody, has proved both highly effective and safe. Read More

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October 2021

Eosinophilia Induced by Blocking the IL-4/IL-13 Pathway: Potential Mechanisms and Clinical Outcomes.

J Investig Allergol Clin Immunol 2022 Jun 6;32(3):165-180. Epub 2022 May 6.

Department of Immunology, IIS-Fundación Jiménez Díaz; CIBER de Enfermedades Respiratorias (CIBERES) and School of Medicine, Universidad Autónoma de Madrid, Madrid, Spain.

Five biological drugs are currently marketed for treatment of uncontrolled severe asthma. They all block type 2 inflammatory pathways by targeting IgE (omalizumab), the IL-5 pathway (mepolizumab, reslizumab, benralizumab), or the IL-4/IL-13 pathway (dupilumab). Hypereosinophilia has been observed in 4%-25% of patients treated with dupilumab and is transient in most cases, although there have been reports of persistent cases of symptomatic hypereosinophilia consistent with eosinophilic granulomatosis with polyangiitis (EGPA), eosinophilic pneumonia, eosinophilic vasculitis, and sudden worsening of asthma symptoms. Read More

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Cutaneous manifestations of acute kidney injury.

Clin Kidney J 2022 May 9;15(5):855-864. Epub 2021 Dec 9.

Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Central Parkway, Newcastle upon Tyne, UK.

Acute kidney injury (AKI) is a common medical problem with a multitude of aetiologies. Prompt diagnosis and management is key in the prevention of complications. Cutaneous signs can often give diagnostic clues of underlying systemic diseases causing AKI. Read More

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Recrudescence of severe polyneuropathy after receiving Pfizer-BioNTech COVID-19 vaccine in a patient with a history of eosinophilic granulomatosis with polyangiitis.

BMJ Case Rep 2022 Apr 29;15(4). Epub 2022 Apr 29.

Internal Medicine, Texas Health Presbyterian Hospital Dallas, Dallas, Texas, USA.

A middle age man with a history of diabetes mellitus type 2, hypertension, migraine and eosinophilic granulomatosis with polyangiitis (EGPA) with polyneuropathy in remission presented with paresthesia and motor weakness soon after receiving the Pfizer-BioNTech COVID-19 messanger RNA (mRNA) vaccine. The patient had polyneuropathy 10 years ago secondary to EGPA, which had resolved. EGPA was diagnosed on the basis of typical symptoms and positive sural nerve biopsy. Read More

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Respiratory involvement in antineutrophil cytoplasmic antibody-associated vasculitides: a retrospective study based on POLVAS registry.

Clin Exp Rheumatol 2022 May 27;40(4):720-726. Epub 2022 Apr 27.

Centre for Intensive Care and Perioperative Medicine, Jagiellonian University Medical College, Kraków, Poland.

Objectives: The study aimed to characterise the Polish population of (ANCA)-associated vasculitides (AAV) with respiratory involvement (RI), in comparison to the subgroup without lung manifestations and the other cohorts.

Methods: Retrospective analysis of the Polish population of AAV with RI was conducted, based on data from the POLVAS registry. Standard descriptive statistics, χ2 test, and Mann-Whitney U test were used to perform comparisons. Read More

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Eosinophilic granulomatosis with polyangiitis-related myocarditis during mepolizumab therapy reveals a Th1/Th17-mediated vasculitic response.

Clin Exp Rheumatol 2022 05 29;40(4):863-864. Epub 2022 Apr 29.

Department of Experimental and Clinical Medicine, University of Firenze, and SOD Interdisciplinary Internal Medicine, Behçet Centre and Lupus Clinic, AOU Careggi Hospital of Florence, Italy.

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Eosinophilic granulomatosis with polyangiitis complicated with rapidly progressive glomerulonephritis in a young man who is a healthy cyclist.

G Ital Nefrol 2022 Apr 21;39(2). Epub 2022 Apr 21.

Nephrology and Dialysis Unit, San Giovanni Evangelista Hospital, Tivoli, Italy.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis. Rapidly progressive glomerulonephritis (RPGN) is a rare complication of EGPA. We report a case of a 60-year-old man, who is also a skilled cyclist, who was hospitalized to investigate a symptomatology that had arisen over the previous months and worsened in the last few weeks, to the point of limiting normal everyday activities. Read More

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Performance of MPO-ANCA and PR3-ANCA immunoassays for the stratification of specific ANCA-associated vasculitis: A systematic review and meta-analysis.

Autoimmun Rev 2022 Jun 19;21(6):103100. Epub 2022 Apr 19.

ARUP Laboratories, Salt Lake City, UT, United States of America; Department of Pathology, University of Utah, Salt Lake City, UT, United States of America. Electronic address:

Objective: To determine the impact of myeloperoxidase (MPO) and proteinase 3 (PR3) antigen-specific immunoassays in the stratification of patients at-risk for anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) at diagnosis.

Methods: A Medline search was conducted to identify diagnostic accuracy studies using PR3-ANCA or MPO-ANCA for the evaluation of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Studies estimates were pooled using the bivariate method. Read More

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Excipient lung disease secondary to intravenous heroin use.

BMJ Case Rep 2022 Apr 21;15(4). Epub 2022 Apr 21.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA

We present a case of a woman who had progressive shortness of breath and wheezing with a mild restrictive pulmonary function pattern. She was initially diagnosed with eosinophilic granulomatosis with polyangiitis on the basis of peripheral eosinophilia, bronchoalveolar lavage eosinophilia (47%) and surgical lung biopsy findings. Six months following her diagnosis, the patient returned because of persistent symptoms, and a second review of the lung biopsy revealed thrombotic lesions in the pulmonary vessels with polarisable foreign body materials, associated giant cell reactions and numerous eosinophil infiltrates, consistent with intravenous drug abuse. Read More

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Therapeutic advances in eosinophilic granulomatosis with polyangiitis.

Curr Opin Rheumatol 2022 05 18;34(3):158-164. Epub 2022 Apr 18.

University of Michigan, Department of Internal Medicine, Division of Rheumatology, Ann Arbor, Michigan, USA.

Purpose Of Review: In recent years, therapeutic advances in eosinophilic granulomatosis with polyangiitis (EGPA) have changed our treatment paradigm. This review will summarize and discuss updates in management of EGPA, with a particular focus on biologic therapies.

Recent Findings: The anti-interleukin (IL)-5 agent mepolizumab (the first FDA-approved drug specifically for EGPA) is effective in induction and maintenance of remission particularly in patients with predominantly asthma and allergic manifestations, though efficacy in ANCA-positive, vasculitic disease is unclear; additional anti-IL-5 agents are under study. Read More

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From Churg-Strauss Syndrome to Eosinophilic Granulomatosis With Polyangiitis: A Historical Review of Nomenclature and Diagnostic Criteria.

Am J Dermatopathol 2022 May;44(5):315-320

Dermatopathologist, Dermatopathology Unit, Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, MA.

Abstract: Eosinophilic granulomatosis with polyangiitis (EGPA) is rare vasculitis syndrome that involves the skin and other organ systems manifesting as asthma, eosinophilia, and pulmonary infiltrates. The understanding of EGPA, previously known as Churg-Strauss Syndrome, has continued to evolve from its earliest documentation in the literature in 1951. Herein, we review key historical advances in the diagnosis, classification, and nomenclature of EGPA that have shaped our understanding of this protean disorder over time. Read More

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Tumefactive eosinophil-rich non-granulomatous small vessel vasculitis in the cerebrum in a patient with idiopathic hypereosinophilic syndrome.

Neuropathology 2022 Jun 11;42(3):239-244. Epub 2022 Apr 11.

Department of Pathology, San-in Rosai Hospital, Yonago, Japan.

The definite diagnosis of central nervous system vasculitis requires pathological verification by biopsy or surgical resection of the lesion, which may not always be feasible. A 74-year-old woman with a history of allergic rhinitis, but not asthma, presented with slowly progressive left hemiparesis. Magnetic resonance imaging of the head revealed a heterogeneously enhancing mass involving the right internal capsule and corona radiata. Read More

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Successful treatment for eosinophilic granulomatosis with polyangiitis causing severe myocarditis followed by cardiac magnetic resonance.

Mod Rheumatol Case Rep 2022 Jun;6(2):248-253

Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan.

A 38-year-old woman had a history of asthma for 20 years. Bullous lesions had appeared on her left side of the back. Two months before admission, the biopsy revealed eosinophilic cellulitis. Read More

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Spontaneous splenic rupture: A rare complication in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

Indian J Dermatol Venereol Leprol 2022 May-Jun;88(3):392-395

Department of Pathology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

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Eosinophilic granulomatosis with polyangiitis: an unusual cause of stroke.

BMJ Case Rep 2022 Apr 5;15(4). Epub 2022 Apr 5.

Division of General Internal Medicine, Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Stroke has become increasingly common with the rise in hyperlipidaemia, hypertension and other metabolic disorders. In this case, a 69-year-old man with several weeks of non-specific symptoms and no history of metabolic disorders presents with unilateral weakness and supraventricular tachycardia. Kidney biopsy confirmed a diagnosis of eosinophilic granulomatosis with polyangiitis. Read More

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A patient with eosinophilic granulomatosis with polyangiitis successfully weaned from corticosteroids through remission induction therapy with mepolizumab.

Mod Rheumatol Case Rep 2022 Jun;6(2):243-247

The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.

The patient was a 74-year-old man who was admitted to our hospital for fever, purpura, abdominal pain, and bilateral numbness. Although the patient tested negative for anti-neutrophil cytoplasmic antibody (ANCA), he presented with an elevated peripheral eosinophil count, increased inflammatory responses, duodenitis, cholecystitis, lung lesions, renal disorder, and peripheral neuropathy. The skin biopsy findings revealed vasculitis. Read More

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Eosinophilic granulomatosis with polyangitis: A new target for biologicals.

Tuberk Toraks 2022 Mar;70(1):93-101

Division of Immunology and Allergy, Department of Chest Diseases, Ankara University Faculty of Medicine, Ankara, Turkey.

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare systemic necrotizing granulomatous vasculitis in the spectrum of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Nevertheless, EGPA has specific clinical, biological and histological properties different from other AAVs [microscopic polyangiitis (MPA) and granulomatous polyangiitis (GPA)]. Recently, thanks to the studies conducted to understand the pathophysiology of EGPA, unlike neutrophils in other AAVs, the main cells involved in EGPA have been observed to be eosinophils. Read More

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Concomitant allergic bronchopulmonary aspergillosis and eosinophilic granulomatosis with polyangiitis after Aspergillus niger infection.

Pulmonology 2022 May-Jun;28(3):231-234. Epub 2022 Mar 28.

Allergy and Clinical Immunology Unit, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; Faculty of Medicine, University of Coimbra, Portugal; ICBR - Coimbra Institute for Clinical and Biomedical Research, CIBB, Faculdade de Medicina, Universidade de Coimbra, Portugal(1)contributed equally.

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Eosinophilic granulomatosis with polyangiitis presenting with repetitive acute coronary syndrome, refractory coronary vasospasm, and spontaneous coronary dissection: a case report.

J Int Med Res 2022 Mar;50(3):3000605221089516

Cardiac Catheterization Laboratory, Department of Cardiology, Sun Yat-sen Memorial Hospital of Sun Yat-sen University, Guangzhou 510120, China.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of eosinophilic vasculitis that is mainly limited to small- and medium-sized arteries. Cardiac involvement is the leading cause of death in patients with EGPA. Spontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome in middle-aged women with no or few traditional cardiovascular risk factors. Read More

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A Case of Eosinophilic Granulomatosis with polyangiitis Presenting with Mononeuritis Multiplex.

Curr Rheumatol Rev 2022 Mar 29. Epub 2022 Mar 29.

Professor and Chairman, Department of Internal Medicine, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.

Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA), also known as Churg Strauss Syndrome, is an uncommon vasculitis associated with antineutrophil cytoplasmic antibody (ANCA). The hallmarks of the disease are asthma, eosinophilia and systemic vasculitis with varying degrees of neurological, cutaneous, cardiac, gastrointestinal and renal involvement. Diagnosisis often difficult since the symptoms are diverse,and a number of differentials need to be excluded. Read More

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