1,232 results match your criteria Churg-Strauss Disease


[Treatment of ANCA-associated vasculitides: Certainties and controversies].

Authors:
Benjamin Terrier

Nephrol Ther 2019 Apr;15 Suppl 1:S7-S12

Service de médecine interne, centre de références des maladies auto-immunes et systémiques rares, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), 24, rue du Faubourg-Saint-Jacques, 75014 Paris, France; Faculté de médecine, université Paris Descartes, 24, rue du Faubourg-Saint-Jacques, 75014 Paris, France. Electronic address:

Antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides are necrotizing vasculitis affecting small blood vessels and include granulomatosis with polyangiitis (formerly Wegener's), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Diagnosis of ANCA-associated vasculitis is based on the clinical presentation, ANCA positivity directed against proteinase 3 or myeloperoxidase, and when possible on histological evidence of vasculitis. Evolution of ANCA-associated vasculitides without treatment leads constantly to death. Read More

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http://dx.doi.org/10.1016/j.nephro.2019.02.008DOI Listing
April 2019
2 Reads

Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation.

Am J Case Rep 2019 Apr 1;20:423-429. Epub 2019 Apr 1.

Department of Cardiology, Odense University Hospital, Odense, Denmark.

BACKGROUND Granulomatosis with polyangiitis (GPA)/Wegener's granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss' syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. Read More

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https://www.amjcaserep.com/abstract/index/idArt/912693
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http://dx.doi.org/10.12659/AJCR.912693DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454581PMC
April 2019
5 Reads

Causes of eosinophilic ascites - a systematic review.

Rom J Intern Med 2018 Dec 1. Epub 2018 Dec 1.

Carol Davila School of Medicine, Bucharest, Romania.

Background: In the last years an uprising interest for a relatively unknown entity, eosinophilic ascites (EA), has been recorded. Our aim is to investigate the potential causes of EA development, as well as clinical, laboratory, endoscopic and radiologic features, management and outcome in these patients.

Methods: The following research was performed on PubMed (MEDLINE) database using the medical subject headings [Mesh] terms "Ascites" AND "Eosinophils". Read More

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http://content.sciendo.com/view/journals/rjim/ahead-of-print
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http://dx.doi.org/10.2478/rjim-2018-0041DOI Listing
December 2018
15 Reads

Immunoglobulin G4-Related Disease Presenting with Clinical Similarity to Churg-Strauss Syndrome.

Isr Med Assoc J 2019 Feb;21(2):124-125

Department of Internal Medicine, Meir Medical Center, Kfar Saba, affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

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February 2019
8 Reads

[Unexpected death due to eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

An Sist Sanit Navar 2019 Feb 5;0(0):67385. Epub 2019 Feb 5.

.

Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg-Strauss syndrome, is a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. Clinically, it presents in various ways and progresses in three phases: prodromic (asthma and rhino-sinusitis), eosinophilic (peripheral eosinophilia and organ involvement), and vasculitic (clinical manifestations due to small vessel vasculitis). The differential diagnosis of EGPA principally includes eosinophilic and vasculitic disorders, early treatment is needed to avoid a fatal outcome. Read More

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http://dx.doi.org/10.23938/ASSN.0395DOI Listing
February 2019
6 Reads

Differential Diagnosis of Churg-Strauss Syndrome with Tingling Sensation and Weakness of the Lower Extremities.

Anesth Pain Med 2018 Dec 28;8(6):e84179. Epub 2018 Nov 28.

Department of Internal Medicine, Division of Rheumatology, Jeju National University Hospital, Jeju, Korea.

Introduction: Churg-Strauss syndrome (CSS; or eosinophilic granulomatosis with polyangiitis) is an extremely rare autoimmune condition characterized by hypereosinophilia and vasculitis of small or medium-sized blood vessels, occurring in patients with asthma and allergic rhinitis.

Case Presentation: A 47-year-old man (height, 175.8 cm and weight, 74. Read More

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http://dx.doi.org/10.5812/aapm.84179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347737PMC
December 2018
5 Reads

Eosinophilic granulomatosis with polyangiitis in a continuous flow left ventricular assist device patient: a case report and review of literature.

Cardiovasc Pathol 2019 Mar - Apr;39:70-73. Epub 2018 Dec 28.

Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, Houston, TX, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare autoimmune disease with an estimated incidence of approximately 0.11 to 2.66 new cases per 1 million people per year and an overall prevalence of 10. Read More

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http://dx.doi.org/10.1016/j.carpath.2018.12.004DOI Listing
December 2018
8 Reads

[Current treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

Authors:
F Moosig J Holle

Z Rheumatol 2019 Jan 9. Epub 2019 Jan 9.

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

For the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) much less data are available when compared to the other anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). At the same time EGPA also differs in many aspects from AAVs. Treatment is guided by the German and international guidelines. Read More

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http://link.springer.com/10.1007/s00393-018-0580-9
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http://dx.doi.org/10.1007/s00393-018-0580-9DOI Listing
January 2019
27 Reads

Mepolizumab as the first targeted treatment for eosinophilic granulomatosis with polyangiitis: a review of current evidence and potential place in therapy.

Ther Clin Risk Manag 2018 7;14:2385-2396. Epub 2018 Dec 7.

School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy,

Mepolizumab is an anti-interleukin-5 (IL-5) humanized monoclonal antibody that binds to free IL-5. It induces bone marrow eosinophil maturation arrest and decreases eosinophil progenitors and subsequent maturation in the blood and bronchial mucosa. Its use has been extensively studied in severe eosinophilic asthma at a dose of 100 mg subcutaneously (SC) every 4 weeks and, more recently, in other hypereosinophilic syndromes. Read More

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https://www.dovepress.com/mepolizumab-as-the-first-targeted-
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http://dx.doi.org/10.2147/TCRM.S159949DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6292233PMC
December 2018
14 Reads

[Hypokinetic cardiac arrest and hypereosinophilia: a case of Kounis or Churg-Strauss syndrome?]

G Ital Cardiol (Rome) 2018 Nov;19(11):655-657

Cardiologia, Ospedale S. Maria della Misericordia, Perugia.

Ischemic heart disease can be caused by multiple factors. However, epidemiological studies have evidenced an association between hypereosinophilia and acute coronary syndrome, most frequently observed in the Kounis and Churg-Strauss syndromes. We here report the case of a 37-year-old man, who was admitted to our hospital for acute coronary syndrome, complicated by hypokinetic cardiac arrest with severe hypereosinophilia. Read More

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http://www.giornaledicardiologia.it/articoli.php?archivio=ye
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http://dx.doi.org/10.1714/3012.30113DOI Listing
November 2018
13 Reads

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss).

Semin Respir Crit Care Med 2018 08 7;39(4):471-481. Epub 2018 Nov 7.

Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Université Paris Descartes, Paris, France.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1669454
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http://dx.doi.org/10.1055/s-0038-1669454DOI Listing
August 2018
27 Reads

Diffuse Alveolar Hemorrhage and Pulmonary Vasculitides: Histopathologic Findings.

Semin Respir Crit Care Med 2018 08 7;39(4):425-433. Epub 2018 Nov 7.

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.

Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1669412
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http://dx.doi.org/10.1055/s-0038-1669412DOI Listing
August 2018
18 Reads

Ultrasound diagnosis of typhlitis.

J Ultrasound 2019 Mar 27;22(1):103-106. Epub 2018 Oct 27.

Department of Radiology-Ospedale del Mare-ASL NA1 Centro-Napoli, Naples, Italy.

Purpose: Typhlitis, also known as neutropenic colitis, is a rare inflammatory condition and a potentially life-threatening disease process that typically involves the cecum. Delay in diagnosis may lead to a fatal prognosis with a death rate of 21-48%. Ultrasound evaluation of right lower quadrant may lead to an accurate and rapid diagnosis. Read More

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http://link.springer.com/10.1007/s40477-018-0333-2
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http://dx.doi.org/10.1007/s40477-018-0333-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430296PMC
March 2019
28 Reads

[Vasculitides and eosinophilic pulmonary diseases].

Z Rheumatol 2018 Dec;77(10):907-922

Abt. für Rheumatologie, Immunologie und Rehabilitation, Kantonsspital St. Gallen, St. Gallen, Schweiz.

Eosinophilic granulocytes form peripheral effector cells controlled by Th2 lymphocytes, which cause local cell, tissue, and functional disorders of infiltrated organs via the release of cytotoxic basic proteins and oxygen radicals. Diseases associated with eosinophilia include systemic and organ-related forms. The lungs are involved in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome), acute and chronic eosinophilic pneumonia, as well as in an organ manifestation in hypereosinophilic syndrome and certain parasitic diseases. Read More

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http://link.springer.com/10.1007/s00393-018-0561-z
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http://dx.doi.org/10.1007/s00393-018-0561-zDOI Listing
December 2018
18 Reads

Coronary artery vasospasm in a patient withChurg-Strauss syndrome.

BMJ Case Rep 2018 Oct 25;2018. Epub 2018 Oct 25.

Westmead Clinical School, University of Sydney, Sydney, New South Wales, Australia.

Patients with Churg-Strauss syndrome often suffer from unusual cardiac manifestations and sudden cardiac death. This differs from patients with other autoimmune disorders, who typically present with premature ischaemic heart disease. We report the case of a 56-year-old man with recurrent coronary vasospasm, including an inferoposterior ST-elevation myocardial infarction, complicated by bradycardic arrest. Read More

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http://dx.doi.org/10.1136/bcr-2018-225321DOI Listing
October 2018
3 Reads

[Clinical analysis of 3 cases of eosinophilic granulomatosis with polyangiitis].

Zhonghua Jie He He Hu Xi Za Zhi 2018 Oct;41(10):783-786

Department of Allergy and Clinical Immunology, State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital of Guangzhou Medical University, Guangzhou 510120, China.

To improve the clinical recognition of eosinophilic granulomatosis with polyangiitis(EGPA) in clinical manifestations, diagnosis and treatment. The clinical manifestations, pathological characteristic, imaging manifestations, diagnosis and the therapy of three patients with EGPA were presented. These 3 patients had asthma-like symptoms and extrapulmonary manifestations of systemic vasculitis. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1001-0939.2018.11.007DOI Listing
October 2018
4 Reads

Severe mitral stenosis secondary to eosinophilic granulomatosis resolving after pharmacological treatment.

Echocardiography 2018 12 18;35(12):2099-2103. Epub 2018 Oct 18.

First Chair and Department of Cardiology, Medical University of Warsaw, Warsaw, Poland.

We present a case of 44-year-old woman who underwent effective pharmacological treatment of severe mitral stenosis. The patient was hospitalized due to rapidly progressive dyspnea. Her medical history included asthma, perennial rhinitis, and nasal polyps. Read More

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http://dx.doi.org/10.1111/echo.14171DOI Listing
December 2018
4 Reads

Serum periostin as a biomarker in eosinophilic granulomatosis with polyangiitis.

PLoS One 2018 11;13(10):e0205768. Epub 2018 Oct 11.

Division of Rheumatology and the Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.

Objective: Identification of a biomarker for disease activity in eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss) remains an unmet need. This study examined the value of serum periostin, a marker of type 2 inflammation, as a measure of disease activity in patients with EGPA.

Methods: Participants enrolled in a multicenter, prospective cohort of patients with EGPA were included in this study if they had disease activity (defined as Birmingham Vasculitis Activity Score [BVAS] > 0) during follow-up. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0205768PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6181402PMC
March 2019
11 Reads
3.230 Impact Factor

Targeted immunotherapy strategies in ANCA-associated vasculitis.

Authors:
Xavier Puéchal

Joint Bone Spine 2018 Sep 7. Epub 2018 Sep 7.

Centre de référence des maladies systémiques auto-immunes rares, département de médecine interne, hôpital Cochin, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France; Université Paris Descartes, 12, rue de l'École-de-Médecine, 75006 Paris, France; Institut Cochin, Inserm U1016, CNRS UMR 8104, Paris, France. Electronic address:

Targeted immunotherapy is substantially improving the management of ANCA-associated vasculitides (AAV), which include granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). This article reviews the current role for targeted immunotherapy in AAV, its validated indications, and avenues for further development. Rituximab is a validated induction treatment for GPA and severe MPA. Read More

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http://dx.doi.org/10.1016/j.jbspin.2018.09.002DOI Listing
September 2018
16 Reads

Eosinophilic Granulomatosis with Polyangiitis: Clinical Pathology Conference and Review.

J Allergy Clin Immunol Pract 2018 Sep - Oct;6(5):1496-1504

UNC Kidney Center, University of North Carolina at Chapel Hill, Chapel Hill, NC.

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a small vessel vasculitis associated with asthma and eosinophilia. Despite its rarity, continuous gains are being made in understanding the disease with knowledge advancements regarding its epidemiology, heterogeneous clinical manifestations, management, and outcomes. Large knowledge gaps remain, however, particularly surrounding pathophysiologic and diagnostic uncertainties. Read More

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http://dx.doi.org/10.1016/j.jaip.2018.07.001DOI Listing
May 2018
15 Reads

Churg-Strauss Syndrome Presenting as Acute Necrotizing Eosinophilic Myocarditis: Concise Review of the Literature.

Curr Hypertens Rev 2019 ;15(1):8-12

Division of Cardiovascular Medicine, University of Southern California, Los Angeles-90033 CA, United States.

Background: Acute eosinophilic myocarditis (EM) is a rare form of heart failure that is characterized by myocardial eosinophilic infiltration usually in association with peripheral eosinophilia. The underlying cause is variable and can include allergic reactions, parasitic infection, idiopathic hypereosinophilic syndrome, malignancy, Loeffler's syndrome, Churg-Strauss syndrome (CSS), early giant cell myocarditis and malignancy. The course is potentially fatal, and early diagnosis and treatment with steroids is essential. Read More

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http://dx.doi.org/10.2174/1573402114666180903164900DOI Listing
January 2019
15 Reads

Severe/uncontrolled asthma and overall survival in atopic patients with eosinophilic granulomatosis with polyangiitis.

Respir Med 2018 09 24;142:66-72. Epub 2018 Jul 24.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. Electronic address:

Background: Although asthma, rhinitis/rhinosinusitis and peripheral eosinophilia are present in virtually all patients with eosinophilic granulomatosis with polyangiitis (EGPA), the role of atopy in these patients is not well defined.

Objective: To clarify the role of atopy in patients affected with EGPA.

Methods: Clinical, laboratory and standard spirometry data have been abstracted from medical records. Read More

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http://dx.doi.org/10.1016/j.rmed.2018.07.017DOI Listing
September 2018
8 Reads

[Vasculitides and eosinophilic pulmonary diseases].

Internist (Berl) 2018 09;59(9):898-910

Abt. für Rheumatologie, Immunologie und Rehabilitation, Kantonsspital St. Gallen, St. Gallen, Schweiz.

Eosinophilic granulocytes form peripheral effector cells controlled by Th2 lymphocytes, which cause local cell, tissue, and functional disorders of infiltrated organs via the release of cytotoxic basic proteins and oxygen radicals. Diseases associated with eosinophilia include systemic and organ-related forms. The lungs are involved in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome), acute and chronic eosinophilic pneumonia, as well as in an organ manifestation in hypereosinophilic syndrome and certain parasitic diseases. Read More

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http://link.springer.com/10.1007/s00108-018-0479-1
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http://dx.doi.org/10.1007/s00108-018-0479-1DOI Listing
September 2018
26 Reads

Eosinophilic Myocarditis Demonstrated Using Cardiac Magnetic Resonance Imaging in a Patient with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Disease).

Cureus 2018 Jun 12;10(6):e2792. Epub 2018 Jun 12.

Department of Cardiology, University of Kansa, Kansas City, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA), historically known as the Churg-Strauss disease, is a small- to medium-sized vessel multi-organ vasculitis with a propensity to involve the heart. EGPA is a rare condition with an estimated annual incidence of one to 4.2 people per million. Read More

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http://dx.doi.org/10.7759/cureus.2792DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6089482PMC
June 2018
8 Reads

Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features.

Postgrad Med 2018 Sep 2;130(7):581-596. Epub 2018 Aug 2.

a Department of Rheumatology and Internal Medicine , Poznan University of Medical Sciences , Poznan , Poland.

Background: Granulomatosis with polyangiitis (GPA) presents a wide spectrum of manifestations from the common respiratory symptoms to infrequent neurological and cardiac complications. The challenge in diagnosis and management makes the rapidly progressive disorder one of the most challenging dilemmas in clinical medicine.

Method: An extensive electronic literature search was done using multiple databases (e. Read More

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http://dx.doi.org/10.1080/00325481.2018.1503920DOI Listing
September 2018
4 Reads

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis: Exuberant Classic Clinical Picture of a Rare Disease.

Case Rep Dermatol 2018 May-Aug;10(2):175-181. Epub 2018 Jun 29.

Sector of Dermatology and Post-Graduation Course - University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

The authors present a classic case of Churg-Strauss syndrome with an exuberant clinical picture in a 34-year-old woman. She showed the following diagnostic criteria: asthma, polyneuropathy, rhinopathy, marked eosinophilia, positive p-ANCA with a perinuclear pattern, and skin histopathology results suggestive of vasculitis with eosinophils. There was a good response to prednisone, dexamethasone pulse therapy, and cyclophosphamide. Read More

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http://dx.doi.org/10.1159/000489162DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6062663PMC
June 2018
6 Reads

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort.

Pediatr Pulmonol 2018 Dec 26;53(12):1640-1650. Epub 2018 Jun 26.

Pediatric Pulmonology and Allergology Department, Hôpitaux pédiatriques de Nice CHU-Lenval, Nice, France.

Objectives: To describe the characteristics of pediatric cases of eosinophilic granulomatosis with polyangiitis (EGPA), a systemic necrotizing vasculitis rarely diagnosed in children, retrieved from the French Reference Center for rare pediatric lung diseases and compared with adult cases included in the French Vasculitis Study Group cohort.

Methods: We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria. Read More

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http://dx.doi.org/10.1002/ppul.24089DOI Listing
December 2018
18 Reads

Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis.

Clin Exp Allergy 2018 Oct 10;48(10):1305-1316. Epub 2018 Jul 10.

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

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a disease characterized by allergic granulomatosis, necrotizing vasculitis, and peripheral blood eosinophilia. Interleukin (IL)-33, thymic stromal lymphopoietin (TSLP), and type 2 innate lymphoid cells (ILC2) are involved in the innate and type 2 immune responses in EGPA. However, the relationships among these molecules and the mechanisms underlying the development of EGPA remain unknown. Read More

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http://dx.doi.org/10.1111/cea.13209DOI Listing
October 2018
15 Reads

Eosinophilic Granulomatosis with Polyangiitis Manifested by Cholecystitis and Mononeuritis Multiplex: A Case Report.

Iran J Med Sci 2018 May;43(3):332-335

Resident in Internal Medicine, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.

Eosinophilic granulomatosis with polyangiitis formerly named "Churg-Strauss syndrome (CSS)" is a systemic disease with bronchial asthma, hypereosinophilia, and systemic vasculitis. We report a case of CSS with cholecystitis and mononeuritis multiplex. A 50-year-old woman with a history of sinusitis and bronchial asthma of 8 years' duration was referred with a complaint of left-hand deformity and difficulty in walking. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5993901PMC
May 2018
6 Reads

Hypereosinophilic syndrome in the differential diagnosis of pulmonary infiltrates with eosinophilia.

Ann Allergy Asthma Immunol 2018 08 24;121(2):179-185. Epub 2018 May 24.

Department of Pathology and Laboratory Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio; Division of Pathology & Laboratory Medicine and Perinatal Institute, Cincinnati Children's Hospital Medical Center, Cincinatti, Ohio.

Objective: To describe key diagnostic considerations in patients with pulmonary infiltrates with eosinophilia, with a special emphasis on raising awareness of hypereosinophilic syndrome (HES), a disease that often involves the lungs and prompts investigation for clonal neoplastic processes that determine prognosis and treatment.

Data Sources: Studies and review articles were selected from PubMed and Scopus for relevance to pertinent topics.

Study Selections: The literature was screened for studies that described lung eosinophilia and HES. Read More

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http://dx.doi.org/10.1016/j.anai.2018.05.014DOI Listing
August 2018
5 Reads

One year in review 2018: systemic vasculitis.

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):12-32. Epub 2018 May 18.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.

Systemic vasculitis are heterogeneous, complex and disabling disorders. Following the previous annual reviews of this series, this paper gives a brief overview on current knowledge about recent literature on small- and large-vessel systemic vasculitis, with a specific focus on pathogenetic and clinical aspects, novel possible disease-related biomarkers and current and future therapies that are in the pipeline. Read More

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July 2018
49 Reads

Mycophenolate mofetil for induction and maintenance of remission in naïve patients with granulomatosis with polyangiitis without renal involvement.

Reumatol Clin 2018 May 16. Epub 2018 May 16.

Faculty of Behavioral, Management and Social sciences, Department Psychology, Health and Technology, University of Twente, Enschede, The Netherlands.

Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up. Read More

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http://dx.doi.org/10.1016/j.reuma.2018.03.007DOI Listing
May 2018
5 Reads

Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review.

Drugs 2018 Jun;78(8):809-821

Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Université Paris Descartes, Paris, France.

Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis. Nevertheless, eosinophilic granulomatosis with polyangiitis stands apart because it has features of vasculitis and eosinophilic disorders that require targeted therapies somewhat different from those used for other anti-neutrophil cytoplasm antibody-associated vasculitides. Considerable advances have been made in understanding the underlying pathophysiology of eosinophilic granulomatosis with polyangiitis that have highlighted the key role of eosinophils and opened new therapeutic opportunities. Read More

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http://dx.doi.org/10.1007/s40265-018-0920-8DOI Listing
June 2018
13 Reads

[Manifestation of eosinophilic granulomatosis with polyangiitis in the head and neck area over time taking systemic disease activity into consideration].

Z Rheumatol 2018 Dec;77(10):928-937

Klinik für HNO-Heilkunde, Kopf- und Halschirurgie, Christian-Albrechts-Universität zu Kiel, Arnold-Heller-Str. 3, Haus 27, 24105, Kiel, Deutschland.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare granulomatous vasculitis of the small and medium sized blood vessels of unknown etiology. A regular involvement of the head and neck area is known. Since it is not yet known whether the inflammatory processes in the head and neck area are parallel to the systemic disease activity, the course of systemic and local disease activity was investigated in a comparative study. Read More

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http://dx.doi.org/10.1007/s00393-018-0439-0DOI Listing
December 2018
26 Reads

Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report.

Clin Rheumatol 2018 Jun 21;37(6):1729-1735. Epub 2018 Mar 21.

Department of Vascular Problems in Rheumatic Diseases, V.A. Nasonova Research Institute of Rheumatology, Kashirskoe shosse 34A, Moscow, Russian Federation, 115522.

Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Read More

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http://dx.doi.org/10.1007/s10067-018-4018-1DOI Listing
June 2018
22 Reads

Hypereosinophilic syndrome presenting acutely with neurologic signs.

Ann Allergy Asthma Immunol 2018 05 28;120(5):461-464. Epub 2018 Feb 28.

Department of Medicine, Division of Clinical Immunology and Allergy, University of Mississippi Medical Center, Jackson, Mississippi.

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http://dx.doi.org/10.1016/j.anai.2018.02.032DOI Listing
May 2018
3 Reads

The initial predictors of death in 153 patients with ANCA-associated vasculitis in a single Korean centre.

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):65-72. Epub 2018 Feb 14.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.

Objectives: We estimated the cumulative patient survival rates, the causes of death and the initial predictors of death in Korean patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA).

Methods: We reviewed the medical records of 153 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We collected clinical and laboratory data including ANCA, Birmingham vasculitis activity score (BVAS), five factor score (FFS) (2009), comorbidities, medications and prognosis (death and relapse). Read More

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July 2018
21 Reads

Churg-Strauss syndrome with cardiac involvement: case illustration and contribution of CMR in the diagnosis and clinical follow-up.

Int J Cardiol 2018 05 3;258:321-324. Epub 2018 Feb 3.

Service de cardiologie, Centre Hospitalier Universitaire Vaudois, Switzerland.

This report summarises three cases of Churg-Strauss syndrome (CSS) illustrating the diagnostic challenges associated with the cardiac manifestation of this disease. Here, we illustrate the role of cardiac magnetic resonance (CMR) for diagnosis and follow-up of CSS with a focus on new non-contrast T-weighted imaging sequences for quantification of myocardial scar tissue and quantitative T mapping techniques, which allow the detection of myocardial edema. Read More

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http://dx.doi.org/10.1016/j.ijcard.2018.02.011DOI Listing
May 2018
8 Reads

Etiology of cutaneous vasculitis: utility of a systemic approach

Gac Med Mex 2018 ;154(1):62-67

Subdirección de Investigación Biomédica, Hospital General Dr. Manuel Gea González, Ciudad de México, México.

Cutaneous vasculities (CV) represents a diagnostic challenge, occurs as primary cutaneous disorder or as a manifestation of other entities.

Objective: To search the cause of CV. Methods: Patients with CV were prospectively evaluated. Read More

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http://dx.doi.org/10.24875/GMM.17002773DOI Listing
June 2018
10 Reads

Epidemiology of primary systemic vasculitis in children: a population-based study from southern Sweden.

Scand J Rheumatol 2018 07 7;47(4):295-302. Epub 2018 Feb 7.

d Department of Rheumatology, Clinical Sciences Lund , Lund University , Lund , Sweden.

Objectives: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden.

Methods: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1. Read More

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http://dx.doi.org/10.1080/03009742.2017.1412497DOI Listing
July 2018
20 Reads

Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients.

Turk Thorac J 2017 Jul 1;18(3):72-77. Epub 2017 Jul 1.

Department of Chest Diseases, Erciyes University School of Medicine, Kayseri, Turkey.

Objectives: Eosinophilic granulomatosis with poliangiitis (EGPA) which was previously called Churg-Strauss Syndrome, is classified into eosinophilic and vasculitic phases. To characterize the eosinophilic and vasculitic phases of the disease in terms of clinical findings, serology, and treatment.

Materials And Methods: We included 15 EGPA patients in the study. Read More

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http://dx.doi.org/10.5152/TurkThoracJ.2017.16040DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5783085PMC
July 2017
9 Reads

Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature.

Int Arch Allergy Immunol 2018 23;175(1-2):61-69. Epub 2018 Jan 23.

Respiratory Pathophysiology Unit, Cardiothoracic and Vascular Department, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. Read More

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http://dx.doi.org/10.1159/000484602DOI Listing
April 2018
15 Reads

Recurrence of eosinophilic granulomatosis with polyangitis after orthotopic heart transplant.

Am J Transplant 2018 Jun 5;18(6):1544-1547. Epub 2018 Mar 5.

Department of Cardiology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Eosinophilic granulomatosis with polyangitis (EGPA), previously referred to as Churg-Strauss syndrome, is a necrotizing small vessel vasculitis associated with eosinophilic infiltrates and extravascular granulomas. We report a case of a Caucasian woman successfully bridged to heart transplantation with a continuous flow left ventricular assist device (LVAD) who survived recurrence of EGPA in the allograft. Read More

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http://dx.doi.org/10.1111/ajt.14679DOI Listing
June 2018
15 Reads

CHARACTERISTICS OF ARTICULAR SYNDROME IN SYSTEMIC VASCULITIS.

Georgian Med News 2017 Dec(273):69-75

Donetsk National Medical University of Health Ministry of Ukraine, Lyman; State Institution "Dnipropetrovsk Medical Academy of the Ministry of Health of Ukraine", Ukraine.

The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1. Joint's damage in the form of arthritis or arthralgia observed in 32-67% different groups of patients, that depending on the disease duration, the degree of the pathological process's activity, extraarticular signs severity, lung parenchyma involving and hemodynamic status in the pulmonary circulation. Read More

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December 2017
10 Reads

Efficacy of omalizumab treatment in a man with occupational asthma and eosinophilic granulomatosis with polyangioitis.

Ann Allergy Asthma Immunol 2018 02 28;120(2):209-211. Epub 2017 Dec 28.

Allergy Unit, Policlinico Agostino Gemelli Hospital, Rome, Italy; IRCCS Oasi Maria SS, Troina, Italy.

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https://linkinghub.elsevier.com/retrieve/pii/S10811206173122
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http://dx.doi.org/10.1016/j.anai.2017.10.034DOI Listing
February 2018
6 Reads

Case Report of Ipilimumab-Induced Diffuse, Nonnecrotizing Granulomatous Lymphadenitis and Granulomatous Vasculitis.

J Pharm Pract 2018 Apr 21;31(2):227-229. Epub 2017 Mar 21.

3 Department of Pharmacy, Levine Cancer Institute, Carolinas HealthCare System, Charlotte, NC, USA.

Ipilimumab is indicated for the treatment of melanoma in both the metastatic and adjuvant setting. Ipilimumab inhibits cytotoxic T-lymphocyte antigen 4, leading to the augmentation of T-cell activity and an antitumor immune system response. The side effect profile of ipilimumab consists of autoimmune-like events such as dermatitis, colitis, and thyroiditis. Read More

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http://dx.doi.org/10.1177/0897190017699762DOI Listing
April 2018
5 Reads

Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis.

An Bras Dermatol 2017 ;92(5 Suppl 1):56-58

Dermatology Outpatient Clinic, Universidade de Mogi das Cruzes - Mogi das Cruzes (SP), Brazil.

Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions. Read More

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http://dx.doi.org/10.1590/abd1806-4841.20175522DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5726678PMC
March 2018
12 Reads

Wells syndrome secondary to influenza vaccination: A case report and review of the literature.

Hum Vaccin Immunother 2018 04 18;14(4):958-960. Epub 2018 Jan 18.

b Division of Internal Medicine, McGill University Health Center , Montreal , Quebec , Canada.

Vaccinations have been shown repeatedly to be extremely safe with low incidence of complications. Given the rarity, these adverse events, they must be reported and examined cautiously. This case report illustrates the first case of an adult presenting with Wells syndrome that developed soon after vaccination with the thiomersal, a common preservative, containing influenza vaccine. Read More

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http://dx.doi.org/10.1080/21645515.2017.1417714DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5893209PMC
April 2018
13 Reads
2.130 Impact Factor