2,669 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis


Omalizumab in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA): single-center experience in 18 cases.

World Allergy Organ J 2018 3;11(1):39. Epub 2018 Dec 3.

1Department of Chest Diseases, Division of Immunology and Allergy, School of Medicine, Ankara University, Ankara, Turkey.

Background: Data are limited regarding the effectiveness of omalizumab in patients with eosinophilic granulomatosis with polyangiitis (EGPA). Our aim was to evaluate the clinical and functional effectiveness of omalizumab in patients with EGPA in long-term follow-up.

Methods: This study was a retrospective chart review of patients with EGPA who were treated with omalizumab injections between May 2012 and April 2018. Read More

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http://dx.doi.org/10.1186/s40413-018-0217-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276141PMC
December 2018

A rare case report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.

BMC Pulm Med 2018 Nov 29;18(1):181. Epub 2018 Nov 29.

Department of Pulmonary, Critical Care, Hyperbaric and Sleep Medicine, Loma Linda University Medical Center, 11234 Anderson St., Loma Linda, CA, 92354, USA.

Background: Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Read More

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http://dx.doi.org/10.1186/s12890-018-0733-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6267840PMC
November 2018
2 Reads

An Atypical Case of Non-asthmatic Eosinophilic Granulomatosis with Polyangiitis Finally Diagnosed by Tissue Biopsy.

Intern Med 2018 Nov 19. Epub 2018 Nov 19.

Department of General Internal Medicine, Hiroshima University Hospital, Japan.

A 78-year-old woman with fever of unknown origin that had persisted for 3 months, systemic edema, and cervical lymphadenopathy was admitted to our hospital. Skin purpura and jaw claudication were subsequently observed. Histopathological examinations of the lymph nodes, skin, and temporal artery revealed findings characteristic of eosinophilic granulomatosis with polyangiitis (EGPA). Read More

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http://dx.doi.org/10.2169/internalmedicine.1167-18DOI Listing
November 2018
3 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
2 Reads

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss).

Semin Respir Crit Care Med 2018 Aug 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
5 Reads

Diffuse Alveolar Hemorrhage and Pulmonary Vasculitides: Histopathologic Findings.

Semin Respir Crit Care Med 2018 Aug 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
8 Reads

Ultrasound diagnosis of typhlitis.

J Ultrasound 2018 Oct 27. 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
October 2018
6 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
9 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
2 Reads

Successful treatment outcomes in pregnant patients with ANCA-associated vasculitides: A systematic review of literature.

Int J Rheum Dis 2018 Sep;21(9):1734-1740

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Aim: Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) are a group of small vessel vasculitis with systemic presentations and considerable morbidity and mortality. Pregnancy in these patients poses a significant therapeutic challenge. There is limited published literature regarding pregnancy in AAV. Read More

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http://dx.doi.org/10.1111/1756-185X.13342DOI Listing
September 2018
1 Read
1.771 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
8 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
6 Reads

Churg-Strauss Syndrome Presenting As Acute Necrotizing Eosinophilic Myocarditis. Concise review of the literature.

Curr Hypertens Rev 2018 Sep 3. Epub 2018 Sep 3.

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

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
September 2018
9 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
14 Reads

Ophthalmic Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome): A Systematic Review of the Literature.

Ophthalmic Plast Reconstr Surg 2018 Aug 21. Epub 2018 Aug 21.

Department of Ophthalmology and Visual Sciences, Montefiore Medical Center, Bronx, New York, U.S.A.

Purpose: To review and summarize the clinical features, presentations, diagnostic modalities, and management of ophthalmic manifestations of eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome).

Methods: A systematic PubMed search of all English articles on EGPA with ophthalmic involvement was performed. Emphasis was placed on English-language articles, but any article with an abstract translated into English was also included. Read More

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http://dx.doi.org/10.1097/IOP.0000000000001202DOI Listing
August 2018
10 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
2 Reads

A very rare case of eosinophilic mastitis.

Int J Surg Case Rep 2018 20;49:251-254. Epub 2018 Jul 20.

Takahashi Breast and Gastroenterology Clinic, Yamazaki Seiren Bldg. 2F, 6-2-22, Uehonmachi, Tennoji-Ku, Osaka City, Osaka 543-0001, Japan. Electronic address:

Introduction: Eosinophilic mastitis caused by eosinophil infiltration of the mammary gland is very rare. To date, no report has been published on treating patients with this disorder using anti-allergic drugs. Steroids are commonly used in these cases, but have greater burden. Read More

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http://dx.doi.org/10.1016/j.ijscr.2018.07.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6080636PMC
July 2018
2 Reads

Histopathological Findings and Increased D-Dimer Are Predictive Factors of Systemic Thromboses in Eosinophilic Granulomatosis With Polyangiitis.

Am J Dermatopathol 2018 Dec;40(12):879-883

Department of Dermatology, Asahikawa Medical University, Asahikawa, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA; ie, Churg-Strauss syndrome) is one of the antineutrophil cytoplasmic antibody-associated vasculitis syndromes. Although extravascular granulomatoses are a well-known histopathological feature, the diverse histopathologic spectrum of cutaneous lesions has not been described in detail. Thus, this study sought to investigate the possible correlation between the clinical features and histopathology of cutaneous lesions in EGPA cases, focusing on systemic thrombogenic conditions, such as visceral infarction and deep vein thrombosis. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001202DOI Listing
December 2018
2 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
10 Reads

Anaesthetic management and the role of sugammadex in a patient with Churg-Strauss syndrome.

Authors:
Ping Han Chia

Indian J Anaesth 2018 May;62(5):400-402

Department of Anaesthesia, Mercy Hospital for Women, Heidelberg Victoria 3084, Australia.

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http://dx.doi.org/10.4103/ija.IJA_19_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5971636PMC
May 2018
2 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
9 Reads

Eosinophilic cellulitis (Wells syndrome) successfully treated with mepolizumab.

JAAD Case Rep 2018 Jul 6;4(6):548-550. Epub 2018 Jun 6.

Department of Dermatology, Division of Immunology, Allergy and Infectious Diseases, Medical University of Vienna, Vienna, Austria.

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http://dx.doi.org/10.1016/j.jdcr.2018.02.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5991892PMC
July 2018
2 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
2 Reads

Eosinophilic granulomatosis with polyangiitis and laryngeal involvement: review of the literature and a cross-sectional prospective experience.

J Laryngol Otol 2018 Jul 11;132(7):619-623. Epub 2018 Jun 11.

Rheumatology Unit,University of Pisa,Italy.

Background: Eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis show variable otorhinolaryngological involvement. Up to 14 per cent of granulomatosis with polyangiitis patients have subglottis involvement; little is known about the laryngeal involvement in eosinophilic granulomatosis with polyangiitis.

Method: A literature review was conducted, together with a prospective cross-sectional analysis of 43 eosinophilic granulomatosis with polyangiitis patients. Read More

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http://dx.doi.org/10.1017/S0022215118000737DOI Listing
July 2018
1 Read

Massive Myocardial Necrosis due to Churg-Strauss Syndrome.

Rev Esp Cardiol (Engl Ed) 2018 Jun 5. Epub 2018 Jun 5.

Servicio de Histopatología, Instituto Nacional de Toxicología y Ciencias Forenses, Barcelona, Spain.

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http://dx.doi.org/10.1016/j.rec.2018.05.007DOI Listing
June 2018
2 Reads

Cardiogenic Shock: An Unusual Initial Presentation of Churg-Strauss Syndrome.

Case Rep Rheumatol 2018 1;2018:2076837. Epub 2018 Apr 1.

Teaching Hospital Jaffna, Faculty of Medicine, University of Jaffna, Jaffna, Sri Lanka.

Churg-Strauss syndrome (CSS) is a rare autoimmune condition, characterized by necrotizing extravascular eosinophil rich granulomatous inflammation of the tissues and disseminated small-medium sized vessel vasculitis in a patient with bronchial asthma and tissue eosinophilia. Though pulmonary involvement is the predominant feature of CSS, extra pulmonary involvement, in particular, cardiac involvement, denotes an adverse outcome. Here we report a 50-year-old female who presented with cardiogenic shock due to an acute coronary event as the initial manifestation of CSS. Read More

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http://dx.doi.org/10.1155/2018/2076837DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5901818PMC
April 2018
7 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
19 Reads

Rate of infections in severe necrotising vasculitis patients treated with cyclophosphamide induction therapy: a meta-analysis.

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):129-134. Epub 2018 May 18.

Division of Rheumatology, Department of Medicine, University of Western Ontario, London, Ontario, Canada.

Objectives: Infections are common complications of necrotising vasculitis. We aimed to determine the rate of infections in patients with severe necrotising vasculitis treated with cyclophosphamide (CYC) combined with high dose glucocorticoids (GC).

Methods: Searches of MEDLINE, Embase and Cochrane Library databases (1990 to May 2016) were performed. Read More

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July 2018
6 Reads

Highlights of the 2nd EUVAS Vasculitis Course.

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):3-11. Epub 2018 May 18.

Nephrology Unit, Parma University Hospital, Parma, Italy.

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July 2018
4 Reads

[Bilateral multifocal choroidal ischemia complicating Churg-Strauss syndrome].

J Fr Ophtalmol 2018 May;41(5):e187-e190

Service d'ophtalmologie, hôpital Omar Drissi, CHU Hassan II, avenue la liberté, Batha, 30110 Fès, Maroc; Faculté de médecine et de pharmacie Fès, université Sidi Mohammed Benabdellah, Fès, Maroc. Electronic address:

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http://dx.doi.org/10.1016/j.jfo.2017.08.026DOI Listing
May 2018
4 Reads

Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review.

Drugs 2018 May 15. Epub 2018 May 15.

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
May 2018
4 Reads

Rise and fall of the eosinophils in heart failure: a rare but important phenomenon seen with cardiomyopathy.

BMJ Case Rep 2018 May 7;2018. Epub 2018 May 7.

Cardiology, University Hospitals Coventry and Warwickshire, Coventry, UK.

A 65-year-old lady and a 69-year-old gentleman, both with a background history of adult-onset asthma, presented with clinical features of heart failure (HF). High-sensitivity cardiac troponin T and eosinophils were significantly raised, along with poor left ventricular (LV) systolic function on cardiac imaging. Endocardial and skin biopsy (in cases 1 and 2, respectively) showed eosinophilic infiltration. Read More

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http://dx.doi.org/10.1136/bcr-2017-221081DOI Listing
May 2018
7 Reads

[Clinical Analysis of 40 Patients with Eosinophilic Lung Diseases in Peking Union Medical College Hospital].

Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2018 Apr;40(2):170-177

Department of Respiratory Medicine,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.

Objective To summarize the clinical features of eosinophilic lung diseases(ELD). Methods We retrospectively analyzed the clinical manifestations,laboratory findings,accessory examination results,and pathology of 40 patients who were diagnosed with ELD and hospitalized in Peking Union Medical College Hospital from January 2013 to December 2016.Results There were 19 males and 21 females,and the average age was(48. Read More

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http://dx.doi.org/10.3881/j.issn.1000-503X.2018.02.006DOI Listing
April 2018
4 Reads

A 92-Year-Old Male with Eosinophilic Asthma Presenting with Recurrent Palpable Purpuric Plaques.

Dermatopathology (Basel) 2018 Jan-Mar;5(1):44-48. Epub 2018 Mar 16.

Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA.

Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis affecting the small and medium-sized vasculature. It is commonly associated with asthma and eosinophilia. Most patients are diagnosed at around the age of 40. Read More

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http://dx.doi.org/10.1159/000485969DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5920959PMC
March 2018
3 Reads

Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis.

Drugs Today (Barc) 2018 Feb;54(2):93-101

Royal Brompton Hospital, London, UK.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare but potentially life-threatening antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis which affects, to varying degrees, the lungs, paranasal sinuses, heart, kidneys, skin and peripheral nervous system. It is strongly associated with asthma. Peripheral eosinophilia is a defining feature of EGPA and eosinophilic inflammation is often observed in biopsies of affected tissues. Read More

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http://dx.doi.org/10.1358/dot.2018.54.2.2788013DOI Listing
February 2018
4 Reads

Eosinophilic Granulomatosis With Polyangiitis: Newer Therapies.

Curr Rheumatol Rep 2018 Apr 2;20(5):23. Epub 2018 Apr 2.

GIRAT: Grupo de Investigación en Reumatología, Autoinmunidad y Medicina Traslacional, Fundación Valle del Lili, Universidad Icesi, Cl 18 #, Cali, 122-135, Colombia.

Purpose Of Review: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic disseminated vasculitis associated with extravascular granulomas in patients suffering from asthma and tissue eosinophilia. Current therapies to achieve remission and prevent relapse include glucocorticoids and immunosuppressants like cyclophosphamide.

Recent Findings: With the right treatment, clinical prognosis is favorable, so concerted efforts have been made in recent years to find new alternatives for treating severe EGPA. Read More

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http://dx.doi.org/10.1007/s11926-018-0736-2DOI Listing
April 2018
10 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
13 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
9 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
15 Reads

Cancer development in Korean patients with ANCA-associated vasculitis: a single centre study.

Clin Exp Rheumatol 2018 Mar-Apr;36 Suppl 111(2):73-77. Epub 2018 Feb 14.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul; and Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Republic of Korea.

Objectives: We investigated the incidence rate and type of cancer, and furthermore, estimated the standardised incidence ratios (SIRs) of cancer in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Korea.

Methods: We retrospectively included 150 patients with AAV [81 patients with microscopic polyangiitis (MPA), 38 with granulomatosis with polyangiitis (GPA) and 31 patients with eosinophilic GPA (EGPA)], and reviewed their medical records. We collected demographic, clinical and laboratory data, and reviewed the use of glucocorticoid and immunosuppressive drugs administered until detection of cancer or last visit. Read More

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July 2018
9 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
4 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
4 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
10 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
5 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
12 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
4 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
7 Reads

Polyps, grommets and eosinophilic granulomatosis with polyangiitis.

J Laryngol Otol 2018 Mar 9;132(3):236-239. Epub 2018 Jan 9.

Department of Otolaryngology, Head and Neck Surgery,University Hospital Limerick,Ireland.

Objective: To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.

Methods: A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis. Read More

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http://dx.doi.org/10.1017/S0022215117002444DOI Listing
March 2018
12 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
4 Reads