2,935 results match your criteria Churg-Strauss Syndrome


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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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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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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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

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 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

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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

Evolution from hypereosinophilic bronchiolitis to eosinophilic granulomatosis with polyangiitis following COVID-19: a case report.

Clin Exp Rheumatol 2021 Jan-Feb;39 Suppl 128(1):11-12. Epub 2021 Jan 13.

Department of Respiratory Medicine, National Coordinating Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, and Claude Bernard University Lyon 1, France.

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[The 484th case:asthma, limb numbness, diplopia, headache].

Zhonghua Nei Ke Za Zhi 2021 Feb;60(2):179-184

Department of Neurology, Second Affiliated Hospital, Fujian Medical University, Quanzhou 362000, China.

A 47-year-old female who had a history of asthma and sinusitis in the past was admitted to hospital with limbs numbness and pain for ten days. The symptoms were aggravated for eight hours. On admission, significant peripheral eosinophilia was noted. Read More

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February 2021

Catastrophic intracranial hemorrhage associated with eosinophilic granulomatous polyangiitis in a patient after Fontan operation.

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

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January 2021

Granulomatosis With Polyangiitis in a Pediatric Male.

Cureus 2020 Dec 13;12(12):e12055. Epub 2020 Dec 13.

Radiology, University of Texas Medical Branch, Galveston, USA.

Significant eosinophilia is a prominent feature in Churg-Strauss syndrome but has not been described in granulomatosis with polyangiitis (GPA) in a pediatric patient. We present a biopsy case that confirmed granulomatosis with polyangiitis with significant eosinophilia > 30% on the initial presentation. Etiologies that could account for eosinophilia were excluded during workup. Read More

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December 2020

Efficacy of plasma exchange for antineutrophil cytoplasmic antibody-associated systemic vasculitis: a systematic review and meta-analysis.

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

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January 2021

Severe biventricular thrombosis in eosinophilic granulomatosis with polyangiitis: a case report.

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

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December 2020

Eosinophilic Cholecystitis Associated with Eosinophilic Granulomatosis with Polyangiitis.

Case Rep Gastroenterol 2020 Sep-Dec;14(3):668-674. Epub 2020 Dec 11.

Department of Gastroenterology, Tokai University Hachioji Hospital, Tokyo, Japan.

We report a case of eosinophilic cholecystitis associated with eosinophilic granulomatosis with polyangiitis (EGPA) complicated by cerebral hemorrhage. A 60-year-old man presented to a local hospital with a diagnosis of acute cholecystitis, with persistent fever and epigastric pain for 2 weeks. His symptoms persisted despite 3-week hospitalization; therefore, he was transferred to our hospital for further evaluation. Read More

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December 2020

The Rash, the Weakness, and the Nephritis: Nerve and Kidney Biopsy Findings in Eosinophilic Granulomatosis With Polyangiitis.

Cureus 2020 Nov 24;12(11):e11676. Epub 2020 Nov 24.

Internal Medicine, Howard University College of Medicine, Washington, DC, USA.

We present a case of eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome in a 66-year Caucasian female who presented with a severe pruritic itch and a progressive upper and lower extremity weakness of unknown duration. The diagnosis of EGPA in this patient remained elusive for an extended period of time due to the absence of respiratory symptoms. In this article, we also discuss the histologic features of EGPA seen in biopsies of the kidney and the nerves and highlight the value they play in diagnosis. Read More

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November 2020

Cardiac involvement in eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): Prospective evaluation at a tertiary referral centre.

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

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Disseminated Nocardia farcinica infection in a patient with EGPA receiving hormonotherapy.

Lancet Infect Dis 2021 01;21(1):148

Department of PET/CT, First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China. Electronic address:

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January 2021

Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis: A systematic literature review.

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

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February 2021