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    An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.
    Saudi J Kidney Dis Transpl 2017 May-Jun;28(3):639-644
    Department of Nephrology, Government Stanley Medical College and Hospital, Chennai, Tamil Nadu, India.
    We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA). Read More

    Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.
    N Engl J Med 2017 05;376(20):1921-1932
    From the Department of Medicine, National Jewish Health, Denver (M.E.W.); the Division of Pulmonary, Critical Care, and Sleep Medicine, University of California San Diego, La Jolla (P.A.); Beth Israel Deaconess Medical Center, Boston (P.A., P.F.W.); the Department of Medicine, University of Cambridge, Cambridge (D.J.), the Department of Orthopaedics, Rheumatology, and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, Oxford (R.L.), Research and Development, Immuno-Inflammation Therapy Area Unit (J.B.), and Research and Development, Statistics, Programming, and Data Standards (S.M.), GlaxoSmithKline, Uxbridge, and Trizell, Oxford (R.P.) - all in the United Kingdom; the Human Eosinophil Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD (P.K., A.K.); the Center for Vasculitis Care and Research, Cleveland Clinic, Cleveland (C.A.L.); the Division of Rheumatology and the Department of Biostatistics and Clinical Epidemiology, University of Pennsylvania (P.A.M.), and the Respiratory Therapy Area Unit and Flexible Discovery Unit, GlaxoSmithKline (J.S.), Philadelphia; Rheumazentrum, Schleswig-Holstein Mitte, Neumünster, Germany (F.M.); the Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN (U.S.); the Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clinic University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona (M.C.C.); the Respiratory Therapeutic Area, GlaxoSmithKline, Research Triangle Park, NC (S.W.Y.); and the Departments of Dermatology and Medicine, University of Utah School of Medicine, Salt Lake City (G.J.G.).
    Background: Eosinophilic granulomatosis with polyangiitis is an eosinophilic vasculitis. Mepolizumab, an anti-interleukin-5 monoclonal antibody, reduces blood eosinophil counts and may have value in the treatment of eosinophilic granulomatosis with polyangiitis.

    Methods: In this multicenter, double-blind, parallel-group, phase 3 trial, we randomly assigned participants with relapsing or refractory eosinophilic granulomatosis with polyangiitis who had received treatment for at least 4 weeks and were taking a stable prednisolone or prednisone dose to receive 300 mg of mepolizumab or placebo, administered subcutaneously every 4 weeks, plus standard care, for 52 weeks. Read More

    Portal Venous Thrombosis: Eosinophilic Vasculitis.
    J Clin Diagn Res 2017 Mar 1;11(3):OD04-OD05. Epub 2017 Mar 1.
    Professor, Department of Internal Medicine/Endocrinology, Pondicherry Institute of Medical Sciences, Puducherry, India.
    Portal Vein Thrombosis (PVT) is caused by various thrombophilic states. PVT secondary to underlying vasculitis especially Churg-Strauss disease is among the rarest presentation. Here, we report a case of peripheral eosinophilia, eosinophilic ascitis and venous thrombosis involving portal vein and superior mesenteric vein diagnosed as Churg-Strauss Syndrome (CSS). Read More

    Two cases of atypical hemolytic uremic syndrome (aHUS) and eosinophilic granulomatosis with polyangiitis (EGPA): a possible relationship.
    CEN Case Rep 2017 May 1;6(1):91-97. Epub 2017 Mar 1.
    Departamento de Medicina Celular y Molecular, Centro de Investigaciones Biológicas (CSIC), Madrid, Spain.
    Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis. Read More

    ANCA-associated vasculitis in childhood: recent advances.
    Ital J Pediatr 2017 May 5;43(1):46. Epub 2017 May 5.
    Nephrology Unit, University Hospital, Parma, Via Gramsci 14, 43126, Parma, Italy.
    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. Read More

    Are the 1990 American College of Rheumatology vasculitis classification criteria still valid?
    Rheumatology (Oxford) 2017 Mar 31. Epub 2017 Mar 31.
    Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.
    Objectives.: Advances in diagnostic techniques have led to better distinction between types of vasculitis, potentially affecting the utility of the 1990 ACR classification criteria for vasculitis. This study tested the performance of these criteria in a contemporary vasculitis cohort. Read More

    Future Prospects of Biologic Therapies for Immunologic Diseases.
    Immunol Allergy Clin North Am 2017 May;37(2):431-448
    Division of Allergy and Immunology, Children's Hospital of Richmond, Virginia Commonwealth University, CHoR Pavilion, 5th Floor, 1000 East Broad Street, Richmond, VA 23298-0225, USA.
    This article presents an overview of future uses for biologic therapies in the treatment of immunologic and allergic conditions. Discussion is centered on the use of existing therapies outside of their current indication or on new therapies that are close to approval. This information may help familiarize practicing allergists and immunologists with therapies they may soon encounter in their practice as well as help identify conditions and treatments that will require further study in the near future. Read More

    Lesson of the month 1: Beware the atypical presentation: eosinophilic granulomatosis with polyangiitis presenting as acute coronary syndrome.
    Clin Med (Lond) 2017 Apr;17(2):180-182
    University Hospital Southampton, Southampton, UK.
    We describe the case of a 45-year-old woman presenting with troponin positive cardiac-sounding chest pain. An initial emergency angiogram demonstrated two vessel coronary disease, including a distal right coronary artery occlusion. No percutaneous coronary intervention was performed and the patient was treated medically. Read More

    Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) as a differential diagnosis of hypereosinophilic syndromes.
    Respir Med Case Rep 2017 8;21:1-6. Epub 2017 Mar 8.
    Department of Pneumology, University Hospital Pedro Ernesto, State University of Rio de Janeiro, Boulevard 28 de Setembro, 77, 2º andar, Vila Isabel, 20551-030, Rio de Janeiro, Brazil; Postgraduate Programme in Medical Sciences, School of Medical Sciences, State University of Rio de Janeiro, Av. Prof. Manoel de Abreu, 444, 2º andar, Vila Isabel, 20550-170, Rio de Janeiro, Brazil.
    Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes. Read More

    Targeting Interleukin-5 or Interleukin-5Rα: Safety Considerations.
    Drug Saf 2017 Mar 20. Epub 2017 Mar 20.
    Allergy and Respiratory Diseases, Department of Internal Medicine, IRCCS AOU San Martino-IST, University of Genoa, Genoa, Italy.
    Asthma is a highly prevalent chronic disease of the airways; approximately 10% of patients with asthma will experience a severe form of the disease. New understanding of the pathogenesis of asthma has enabled the development of novel drugs and provided hope for patients with asthma. Interleukin (IL)-5 and IL-5 receptor subunit α (IL-5-Rα) plays a crucial role in the development, maturation, and operation of eosinophils so were the first important therapeutic target of these new drugs. Read More

    Hospital Morbidity Database for Epidemiological Studies on Churg-Strauss Syndrome.
    Adv Exp Med Biol 2017 Mar 3. Epub 2017 Mar 3.
    Department of Social Medicine and Public Health, Medical University of Warsaw, Oczki Str. 3, 02-007, Warsaw, Poland.
    Churg-Strauss syndrome or more accurately eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel necrotizing vasculitis with a characteristic late-onset allergic rhinitis and asthma. The use of hospital morbidity database is an important element of the epidemiological analysis of this rare disease. The present study was undertaken to assess the incidence of EGPA and factors related to its epidemiology in Poland; the first analysis of the kind in Poland, enabling a comparison in the European context. Read More

    Multiple perforations and fistula formation following corticosteroid administration: A case report.
    World J Clin Cases 2017 Feb;5(2):67-72
    Jing-Ni He, Zhong Tian, Xu Yao, Yun Yu, Yuan Liu, Department of General Surgery, Shengjing Hospital Affiliated to China Medical University, Shenyang 110000, Liaoning Province, China.
    Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We report a patient with EPGA treated with high-dose steroid who underwent emergency surgery for intestinal perforations. Read More

    Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Hepatitis B Infection - A Rare Association.
    J Clin Diagn Res 2016 Dec 1;10(12):OD07-OD09. Epub 2016 Dec 1.
    Senior Resident, Department of Medicine, Himalayan Institute of Medical Sciences , Dehradun, Uttarakhand, India .
    Eosinophilic Granulomatosis with Polyangiitis (EGPA) or Churg-Strauss syndrome is a rare systemic illness which is characterized by necrotizing inflammation of small and medium sized vessels. The prominent features include asthma, eosinophilia, transient pulmonary infiltration and systemic vasculitis. Various triggering factors have been reported as putative aetiological agents of Churg-Strauss syndrome. Read More

    Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
    Int J Cardiol 2017 Jun 1;236:151-156. Epub 2017 Feb 1.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background-aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis.

    Patients-methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Read More

    Eosinophilic endomyocardial disease.
    Cardiovasc Pathol 2017 Mar - Apr;27:54-56. Epub 2017 Jan 23.
    Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, 6431 Fannin, MSB 1.150, Houston, TXs 77030, United States.
    Eosinophilic granulomatosis with polyangiitis is the rarest of the antineutrophil cytoplasmic antibody-associated vasculitides. We describe the case of a 58-year-old woman with a history of asthma found to have a left ventricular apical thrombus. Cardiac catheterization with endomyocardial biopsy was consistent with eosinophilic endomyocardial disease. Read More

    Heart failure not responsive to standard immunosuppressive therapy is successfully treated with high dose intravenous immunoglobulin therapy in a patient with Eosinophilic Granulomatosis with Polyangiitis (EGPA).
    Int Immunopharmacol 2017 Apr 31;45:13-15. Epub 2017 Jan 31.
    Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy.
    Glucocorticoids and immunosuppressive drugs represent the first-line treatment of eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss syndrome), even though the combined therapy is not successful in achieving the disease remission in some patients with neurological or cardiac involvement. We describe a case of an EGPA male patient with impaired left ventricular function not responsive to glucocorticoid and immunosuppressive therapy. We observed that high-dose (2g/kg/4weeks) intravenous immunoglobulin (IVIG) therapy significantly improved cardiac function, which was deteriorated after reducing IVIG dose at 0. Read More

    ANCA-associated vasculitis.
    Clin Med (Lond) 2017 Feb;17(1):60-64
    Ipswich Hospital and honorary senior lecturer Norwich Medical School, University of East Anglia, Norwich, UK.
    The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome). Read More

    A pragmatic approach to vasculitis in the gastrointestinal tract.
    J Clin Pathol 2017 Jun 24;70(6):470-475. Epub 2017 Jan 24.
    Department of Pathology, Laboratory Medicine Program, University Health Network and University of Toronto, Toronto, Ontario, Canada.
    Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. Read More

    Autoimmune hepatitis and eosinophilic granulomatosis with polyangiitis: a rare association.
    BMJ Case Rep 2017 Jan 20;2017. Epub 2017 Jan 20.
    Department of Internal Medicine, Reading Health System, Sixth Avenue and Spruce Street, West Reading, Pennsylvania, USA.
    We describe a case of 40-year-old woman who presented to our hospital with symptoms of non-radiating epigastric pain for 4-5 days associated with nausea and vomiting. Her history was significant for asthma with recurrent exacerbations, polyneuropathy and recurrent sinus infections. Liver function tests revealed cholestasis. Read More

    Eye problems in a woman with Churg-Strauss syndrome.
    JAAPA 2017 Feb;30(2):24-27
    Benjamin D. Pierce practices otolaryngology at the Mayo Clinic in Phoenix, Ariz. Carrlene B. Donald practices in the Department of Otolaryngology and Head/Neck Surgery at the Mayo Clinic in Phoenix, Ariz. Anthony Mendez is an instructor in the Department of Otolaryngology and Head/Neck Surgery at the Mayo Clinic College of Medicine in Phoenix and director of the PA fellowship in otolaryngology. The authors have disclosed no potential conflicts of interest, financial or otherwise.
    Churg-Strauss syndrome is a rare, systemic vasculitis of unknown cause. Ocular involvement is a rare but established complication and can lead to vision damage or blindness if not treated promptly. Treatment of ocular manifestations corresponds with systemic treatment of the disease and consists primarily of corticosteroids. Read More

    Disorders characterized by predominant or exclusive dermal inflammation.
    Semin Diagn Pathol 2017 May 14;34(3):273-284. Epub 2016 Dec 14.
    Section of Dermatopathology, Division of Surgical Pathology & Cytopathology, University of Virginia Medical Center, Room 3020 University of Virginia Hospital, 1215 Lee Street, Charlottesville, VA 22908-0214, USA. Electronic address:
    Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Read More

    Low Median Nerve Palsy as Initial Manifestation of Churg-Strauss Syndrome.
    J Hand Surg Am 2017 Jan 11. Epub 2017 Jan 11.
    Department of Orthopaedic Surgery, Seoul National University College of Medicine, Seoul, Korea.
    Anterior interosseous nerve (AIN) syndrome is typically characterized by forearm pain and partial or complete dysfunction of the AIN-innervated muscles. Although the exact etiology and pathophysiology of the disorder remain unclear, AIN syndrome is increasingly thought to be an inflammatory condition of the nerve rather than a compressive neuropathy because the symptoms often resolve spontaneously following prolonged observation. However, peripheral neuropathy can be 1 of the first symptoms of systemic vasculitis that needs early systemic immunotherapy to prevent extensive nerve damage. Read More

    Clinical Characteristics and Treatment Response of Peripheral Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center.
    J Clin Neurol 2017 Jan;13(1):77-83
    Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
    Background And Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA.

    Methods: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Read More

    ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.
    J Stroke Cerebrovasc Dis 2017 Mar 5;26(3):e47-e49. Epub 2017 Jan 5.
    Stroke Unit, Mediterraneo Hospital, Athens, Greece.
    Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Read More

    Paediatric anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: an update on renal management.
    Pediatr Nephrol 2017 Jan 6. Epub 2017 Jan 6.
    Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
    The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening. Read More

    A Rare Right Ventricular Involvement of Eosinophilic Granulomatosis Demonstrated by Cardiac MRI.
    Pol J Radiol 2016 13;81:598-601. Epub 2016 Dec 13.
    Department of Clinical Imaging, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
    Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis with a prevalence rate of seven per million. Cardiac involvement was reported in 20-50%, yet with improved diagnostic methods, the frequency of cardiac involvement is expected to be even higher. It can result in significant morbidity and mortality, accounting for about 50% of death. Read More

    Clinical and Economic Burden of Elevated Blood Eosinophils in Patients With and Without Uncontrolled Asthma.
    J Manag Care Spec Pharm 2017 Jan;23(1):85-91
    4 Teva Pharmaceuticals, Frazer, Pennsylvania.
    Background: The European Respiratory Society and American Thoracic Society (ERS/ATS) published guidelines in 2014 for the evaluation and treatment of asthma. These guidelines draw attention to management of patients with asthma that remains uncontrolled despite therapy. One phenotypic characteristic of therapy-resistant asthma is eosinophil elevation. Read More

    A case report and literature review of Churg-Strauss syndrome presenting with myocarditis.
    Medicine (Baltimore) 2016 Dec;95(51):e5080
    Department of Cardiology, The Second Affiliated Hospital, Xi'an Jiaotong University School of Medicine, Xi'an, Shaanxi, P.R. China.
    Background: Churg-Strauss syndrome (CSS) is a multisystem disorder characterized by asthma, prominent peripheral blood eosinophilia, and vasculitis signs.

    Case Summary: Here we report a case of CSS presenting with acute myocarditis and heart failure and review the literature on CSS with cardiac involvement. A 59-year-old man with general fatigue, numbness of limbs, and a 2-year history of asthma was admitted to the department of orthopedics. Read More

    Multiple Thromboembolic Cerebral Infarctions from the Aorta in a Patient with Churg-Strauss Syndrome.
    J Stroke Cerebrovasc Dis 2017 Feb 16;26(2):e32-e33. Epub 2016 Dec 16.
    Department of Neurological Surgery, Wakayama Rosai Hospital, Wakayama, Japan. Electronic address:
    Background: Ischemic stroke is a rare complication of Churg-Strauss syndrome (CSS) and its pathogenesis has not been well clarified yet. We report a case of cerebral infarction in a patient with CSS due to embolism from a thrombus on the wall of the aorta.

    Case: A 39-year-old man had multiple cerebral infarctions with symptoms of mild left hemiparesis and reduced vision. Read More

    Acute coronary vasospasm in a patient with eosinophilic granulomatosis with polyangiitis following NSAID administration: A case report.
    Medicine (Baltimore) 2016 Nov;95(47):e5259
    Internal Medicine, Angers University Hospital, Angers, France.
    Eosinophilic with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) is a rare systemic disease characterized by a small-vessel necrotizing vasculitis. Cardiac manifestations are broad-ranging and are associated with a poor prognosis. Coronary vasospasm is uncommon. Read More

    Eosinophilic Granulomatosis with Polyangiitis Presenting with Skin Rashes, Eosinophilic Cholecystitis, and Retinal Vasculitis.
    Am J Case Rep 2016 Nov 18;17:864-868. Epub 2016 Nov 18.
    Zhongshang Ophthalmic Center, Sun Yat-sen University, Guangzhou, Guangdong, China (mainland).
    BACKGROUND Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare vasculitis of unknown etiology. Most of the patients have a long history of asthma and then develop autoimmune inflammation of small and medium-sized blood vessels, with consequent reduction of blood flow to various organs and tissues. EGPA can cause disorders in multiple systems; the most seriously affected organs are the retina, kidney, brain, cardiovascular system, and skin. Read More

    [The Lymphoid Variant of HES (L-HES) as Differential Diagnose of Severe Asthma in Childhood].
    Klin Padiatr 2016 Nov 15;228(6-07):319-324. Epub 2016 Nov 15.
    Children's Hospital, University of Würzburg, Würzburg.
    Based on a case report an overview on the differential diagnostic considerations with respect to blood hypereosinophilia (HE) and hypereosinophilic syndromes (HES) in childhood is given. A 13-year-old boy was admitted for the clarification of an asthma. In the blood count an increased HE with 3 500/µl (30%) was found along with elevated total serum IgE and IL-5 level (2 000 IU/ml and 17 pg/ml). Read More

    Native T1 Mapping Demonstrating Apical Thrombi in Eosinophilic Myocarditis Associated with Churg-Strauss Syndrome.
    Korean Circ J 2016 Nov 1;46(6):882-885. Epub 2016 Nov 1.
    Department of Radiology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University, Seoul, Korea.
    Eosinophilic myocarditis is a disease characterized by eosinophilic infiltration of the myocardium, consisting of acute necrotic stage, thrombotic stage, and fibrotic stage. Although T1 mapping has been increasingly used in various cardiac pathologies, there has been no report of T1 mapping in eosinophilic myocarditis. We report a case of 75-year-old female with eosinophilic myocarditis, whose cardiac magnetic resonance imaging included native T1 mapping, in which apical thrombi were distinctly seen as areas with decreased T1 values, next to areas of inflammation seen as increased T1 value in subendocardium. Read More

    Radiological and Clinical Features of Eosinophilic Granulomatosis with Polyangiitis.
    Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2016 Oct;38(5):617-620
    Department of Radiology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
    Eosinophilic granulomatosis with polyangiitis(EGPA),also known as Churg-Strauss syndrome,is a clinically rare small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs),characterized by asthma,disseminated necrotizing vasculitis,extravascular granulomas,peripheral eosinophilia,and tissue eosinophilia. This article reviews the pathology,imaging,and clinical features of EGPA. Read More

    Head and Neck Manifestations of Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review.
    Otolaryngol Head Neck Surg 2016 Nov 28;155(5):771-778. Epub 2016 Jun 28.
    Department of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
    Objective: To conduct the first and only systematic review of the existing literature on head and neck manifestations of eosinophilic granulomatosis with polyangiitis to guide clinical decision making for the otolaryngologist.

    Data Sources: PubMed, Cochrane Library, Scopus, and LILACS.

    Review Methods: A systematic review of the aforementioned sources was conducted per the PRISMA guidelines. Read More

    Novel targets of omalizumab in asthma.
    Curr Opin Pulm Med 2017 Jan;23(1):56-61
    aUniv Paris-Sud, Faculté de Médecine, Université Paris-Saclay bAP-HP, Service de Pneumologie, Hôpital Bicêtre cAPHP, Service de Physiologie, Explorations Fonctionnelles Respiratoires, Hôpital Bicêtre, Le Kremlin-Bicêtre dInserm UMR_S 999, Hôpital Marie Lannelongue, Le Plessis-Robinson, France.
    Purpose Of Review: Omalizumab is a recombinant humanized anti-IgE monoclonal antibody approved in the US for moderate to severe persistent allergic asthma (severe persistent asthma in the European Union), uncontrolled despite treatment with inhaled corticosteroids and long-acting beta2 agonists. It reduces asthma exacerbations, symptoms, oral corticosteroid doses, and improves quality of life.

    Recent Findings: Omalizumab may have an antiviral effect when used as a preventive therapy for fall exacerbations in children and teenagers. Read More

    Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
    Autoimmun Rev 2017 Jan 23;16(1):1-9. Epub 2016 Sep 23.
    Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre national de référence des maladies pulmonaire rares, Université de Lyon, Université Claude Bernard Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon, France.
    Objective: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA.

    Methods: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease.

    Results: The study population included 157 patients (mean age 49. Read More

    Churg-Strauss syndrome associated with antiphospholipid antibodies in a patient with retinal vasculitis.
    Arch Soc Esp Oftalmol 2016 Nov 9;91(11):547-550. Epub 2016 Apr 9.
    Servicio de Oftalmología, Hospital Universitario Virgen del Rocío, Sevilla, España.
    Case Report: We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Read More

    Dermatologic uses of omalizumab.
    J Dermatolog Treat 2016 Nov 7:1-6. Epub 2016 Nov 7.
    a Division of Dermatology, Department of Medicine , University of Calgary , Calgary , Alberta , Canada.
    Purpose: Omalizumab is a recombinant humanized monoclonal antibody that inhibits the binding of immunoglobulin E (IgE) to the high-affinity IgE receptor (FceRI) on the surface of mast cells and basophils. Omalizumab has been approved for use in asthma, and new reports show promise in a variety of dermatologic diseases. Herein, we review the literature on omalizumab in dermatology and discuss the safety, efficacy and mechanisms of action for this emerging therapy. Read More

    Updates in ANCA-associated vasculitis.
    Eur J Rheumatol 2016 Sep 29;3(3):122-133. Epub 2016 Jan 29.
    Department of Medicine, Division of Rheumatology, Vasculitis Clinic, Mount Sinai Hospital, University Health Network, University of Toronto, Ontario, Canada.
    Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. Read More

    Echocardiographic Changes in Eosinophilic Endocarditis Induced by Churg-Strauss Syndrome.
    Intern Med 2016;55(19):2819-2823. Epub 2016 Oct 1.
    Department of Cardiovascular Medicine, National Defense Medical College, Japan.
    Eosinophilic myocarditis may be accompanied by Churg-Strauss syndrome (CSS). We report a case of CSS that was accompanied by myocardial changes in the early stage. A 71-year-old woman complained of mild chest pain at rest, but routine echocardiography did not reveal any endocardial abnormalities. Read More

    Imaging of Eosinophilic Lung Diseases.
    Radiol Clin North Am 2016 Nov 12;54(6):1151-1164. Epub 2016 Aug 12.
    Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 1478, Houston, TX 77030, USA. Electronic address:
    Eosinophilic lung diseases encompass a broad range of conditions wherein patients present with pulmonary opacities and eosinophilia of the serum, pulmonary tissue, or bronchoalveolar lavage fluid. Many of these entities can be idiopathic or are secondary to parasitic infection, exposure to drugs, toxins, or radiation. These diseases exhibit a wide range of imaging findings, including consolidation, ground-glass opacities, nodules, and masses. Read More

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