2,686 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis


[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.

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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
1 Read

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
1 Read

Yield of Screening Tests for Systemic Vasculitis in Young Adults with Ischemic Stroke.

Eur Neurol 2019 Feb 4;80(5-6):245-248. Epub 2019 Feb 4.

Department of Neurology, Inha University School of Medicine, Incheon, Republic of

Systemic vasculitis, which can involve the brain, may be one of the causes of stroke in young adults; therefore, a test panel for systemic vasculitis is considered for some young stroke patients. However, little is known about this test's yield as a screening test in young adults with ischemic stroke. We evaluated the yield of a panel for systemic vasculitis as a screening test in young patients with ischemic stroke. Read More

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http://dx.doi.org/10.1159/000496373DOI Listing
February 2019
1 Read
1.362 Impact Factor

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

Cardiovasc Pathol 2018 Dec 28;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
1 Read

[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
5 Reads

Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis.

J Allergy Clin Immunol 2018 Dec 19. Epub 2018 Dec 19.

Department of Medicine, National Jewish Health, Denver, Colo. Electronic address:

Background: In a recent phase III trial (NCT02020889) 53% of mepolizumab-treated versus 19% of placebo-treated patients with eosinophilic granulomatosis with polyangiitis (EGPA) achieved protocol-defined remission.

Objective: We sought to investigate post hoc the clinical benefit of mepolizumab in patients with EGPA using a comprehensive definition of benefit encompassing remission, oral glucocorticoid (OGC) dose reduction, and EGPA relapses.

Methods: The randomized, placebo-controlled, double-blind, parallel-group trial recruited patients with relapsing/refractory EGPA receiving stable OGCs (prednisolone/prednisone, ≥7. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00916749183278
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http://dx.doi.org/10.1016/j.jaci.2018.11.041DOI Listing
December 2018
6 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
8 Reads

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
2 Reads

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
3 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
5 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
8 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
12 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
11 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
14 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
14 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
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
3 Reads
1.771 Impact Factor

Churg-Strauss vasculitis presenting with steroid-responsive left ventricular cardiac mass.

BMJ Case Rep 2018 Oct 17;2018. Epub 2018 Oct 17.

Security Forces Hospital Program, Division of Rheumatology, Department of Internal Medicine, Riyadh, Saudi Arabia.

A 35-year-old black Saudi man, with a known case of bronchial asthma and allergic rhinitis since childhood, presented with joint pain and swelling, orthopnoea, paroxysmal nocturnal dyspnoea and lower extremity oedema. On examination, we found jugular venous distension, bilateral basal crepitation, wheezing and diffuse synovitis. Investigations were notable for peripheral blood eosinophilia, pericardial effusion and elongated structure in the left ventricular outflow tract on echocardiography, mediastinal and hilar lymphadenopathy and right upper lobe infiltrate on high-resolution CT scan. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22605
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http://dx.doi.org/10.1136/bcr-2018-226052DOI Listing
October 2018
11 Reads

A Surprising Cause for Intestinal Obstruction.

Gastroenterology 2018 12 8;155(6):e1-e3. Epub 2018 Sep 8.

D'Or Institute for Research and Education, Rio de Janeiro, Brazil.

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http://dx.doi.org/10.1053/j.gastro.2018.08.052DOI Listing
December 2018
5 Reads

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
12 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
12 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
14 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
6 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
20 Reads

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

Ophthalmic Plast Reconstr Surg 2019 Jan/Feb;35(1):7-16

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
14 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
3 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
4 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
18 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
3 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
3 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
4 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
6 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
8 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
32 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
7 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
6 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 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
7 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
13 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
6 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
7 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
6 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
11 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
16 Reads