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    2508 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis

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    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 Feb 1. 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

    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 Jan 30;45:13-15. Epub 2017 Jan 30.
    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 Jan 24. 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

    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 2016 Dec 14. 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

    Think twice - Diagnostic delay in a patient with acute chest pain.
    Respir Med Case Rep 2016 20;19:94-7. Epub 2016 Aug 20.
    Department of Lung medicine, Bispebjerg University Hospital, Denmark.
    Heart involvement is the most critical and potentially lethal systemic manifestation in eosinophilic granulomatosis with polyangiitis (EGPA). We present a case of acute chest pain in a 58-year-old male with severe asthma, which regressed after sublingual administration of nitroglycerine. At the time of hospital admission, there were non-specific ST-changes on the ecg, coronary enzymes were increased, and the patient was concluded to have a non-ST-elevation myocardial infarction, and treated as such. Read More

    A Diagnostic Algorithm for Eosinophilic Granulomatosis with Polyangiitis Initially Diagnosed as Lumbar Disc Hernia or Lumbar Spinal Stenosis: Personal Experience and Review of the Literature.
    Acta Med Okayama 2016 Aug;70(4):261-8
    Department of Orthopaedic Surgery, Yokohama Rosai Hospital, Yokohama 222-0036,
    Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare systemic vasculitis and is difficult to diagnose. EGPA has a number of symptoms including peripheral dysesthesia caused by mononeuropathy multiplex, which is similar to radiculopathy due to lumbar disc hernia or lumbar spinal stenosis. Therefore, EGPA patients with mononeuropathy multiplex often visit orthopedic clinics, but orthopedic doctors and spine neurosurgeons have limited experience in diagnosing EGPA because of its rarity. Read More

    Eosinophilic Lung Diseases.
    Clin Chest Med 2016 09 25;37(3):535-56. Epub 2016 Jun 25.
    Hospices Civils de Lyon, Louis Pradel Hospital, National Reference Center for Rare Pulmonary Diseases, Department of Respiratory Diseases, F-69677 Lyon, France; Univ Lyon, Université Lyon I, INRA, UMR754, 8 avenue Rockefeller, F-69008 Lyon, France. Electronic address:
    Eosinophilic lung diseases especially comprise eosinophilic pneumonia or as the more transient Löffler syndrome, which is most often due to parasitic infections. The diagnosis of eosinophilic pneumonia is based on characteristic clinical-imaging features and the demonstration of alveolar eosinophilia, defined as at least 25% eosinophils at BAL. Peripheral blood eosinophilia is common but may be absent at presentation in idiopathic acute eosinophilic pneumonia, which may be misdiagnosed as severe infectious pneumonia. Read More

    Not All Coughs Are Asthma or Allergies: Churg-Strauss Syndrome.
    J Emerg Med 2016 Sep 16;51(3):305-7. Epub 2016 Jul 16.
    Department of Family Medicine, Southside Hospital, Bay Shore, New York.
    Background: Cardiomyopathy in patients with Churg-Strauss syndrome (CSS) carries a poor prognosis, with a high 5-year mortality rate, and requires treatment with immunosuppressive therapy. There is no single pathognomonic test or clinical finding for diagnosing CSS; instead, it is based upon meeting four of six criteria.

    Case Report: A 47-year-old woman with a 6-month medical history of "asthma" presented to our hospital with a 1-month history or dyspnea on exertion. Read More

    The significance of early screening with echocardiography in eosinophilic granulomatosis with polyangiitis.
    J Med Ultrason (2001) 2016 Oct 2;43(4):527-31. Epub 2016 Jul 2.
    Department of Laboratory Medicine, School of Medicine, Keio University, Tokyo, Japan.
    Löffler endocarditis is a serious complication of idiopathic hypereosinophilic syndrome characterized by a peculiar type of fibrosing endocarditis. It results in heart failure due to restrictive cardiomyopathy and systemic embolism due to intracardiac thrombus. Here we present a case of a 57-year-old man with Löffler endocarditis concomitant with eosinophilic granulomatosis with polyangiitis. Read More

    [Current approaches to diagnosing and treating eosinophilic granulomatosis with polyangiitis: The 2015 international guidelines].
    Ter Arkh 2016 ;88(5):86-92
    V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
    The 2015 international guidelines for the diagnosis and treatment of eosinophilic granulomatosis with polyangiitis, formerly known as the Churg-Strauss syndrome, are detailed and an attempt is made to expand an evidence base, by attracting more recent available publications. The new guidelines should not be regarded as final standards; these are primarily to extend the possibilities of choosing a personified management strategy for patients with eosinophilic granulomatosis with polyangiitis and to serve as the starting point for further in-depth investigations. Read More

    Optimal therapy and prospects for new medicines in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
    Expert Rev Clin Immunol 2016 Oct 6;12(10):1059-67. Epub 2016 Jun 6.
    c Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), Hôpital Cochin, APHP , Université Paris Descartes , Paris , France.
    Introduction: The prevalence of eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome) is lower than that of other antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV's), and only a few randomized controlled trials have been conducted for this rare disease. However, recent international efforts have helped delineate the best treatment approach.

    Areas Covered: At present, EGPA conventional therapy is by default similar to that of other AAVs. Read More

    Acute Lid Swelling: a Series of Unusual Cases, Treatment and Follow-up.
    Klin Monbl Augenheilkd 2016 Apr 26;233(4):402-5. Epub 2016 Apr 26.
    Department of Ophthalmology, University Hospital Zurich, Zürich, Switzerland (Chairman: Prof. Dr. K. Landau).
    Background: There are many different aetiologies for acute lid swelling, including infection, inflammation, degeneration, tumours and trauma. We present five uncommon cases of acute lid swelling and give an overview of differential diagnoses for rapidly evolving eyelid swelling.

    History And Signs: We reviewed the charts of five patients with initial presentation of acute lid swelling. Read More

    Unsolved questions and concerns about treatment of anti-neutrophil cytoplasm antibody-associated vasculitides.
    Clin Exp Rheumatol 2016 May-Jun;34(3 Suppl 97):S121-8. Epub 2016 Apr 13.
    Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, INSERM U1016, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, University of Paris Descartes, Paris, France.
    The treatment strategy for vasculitis has changed dramatically over the last few years, but some major questions remain. Herein, after reviewing the literature, we provide answers to, or at least an analysis of, available evidence on 10 specific and practical questions concerning ANCA-associated vasculitis management. Read More

    Focus on audiologic impairment in eosinophilic granulomatosis with polyangiitis.
    Laryngoscope 2016 Dec 14;126(12):2792-2797. Epub 2016 Apr 14.
    Rheumatology Unit, University of Pisa, Pisa, Italy.
    Objectives/hypothesis: To evaluate the clinical features of audiologic impairment and its relationship with the nasal, vestibular, and rheumatologic profile in a cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome.

    Study Design: Prospective cross-sectional study.

    Methods: Thirty-nine patients with EGPA, considered controlled according to the Birmingham Vasculitis Activity Score and the Vasculitis Damage Index, underwent a complete audiologic evalutaion with otomicroscopy, impedance audiometry, speech audiometry, and auditory brainstem responses; rhinologic evaluation was made by means of fiberoptic endoscopy and nasal cytology; the clinical evaluation was completed with analysis of the facial function and, in patients with referred vertigo, with videonystagmography (VNG). Read More

    Deposition of elafin in the involved vascular wall of neutrophil-mediated cutaneous vasculitis.
    J Eur Acad Dermatol Venereol 2016 Sep 6;30(9):1544-9. Epub 2016 Apr 6.
    Department of Dermatology, National Defense Medical College, Saitama, Japan.
    Background: Neutrophil elastase plays an important role in skin inflammation induced by neutrophil infiltration. Elafin is an inducible elastase inhibitor expressed by keratinocytes, and is known to be involved in pathogenesis of neutrophilic skin disorders such as psoriasis.

    Methods: Immunohistochemical studies of elafin expression in the cases of vasculitis were performed. Read More

    Successful Anti-PD-1 Antibody Treatment in a Metastatic Melanoma Patient With Known Severe Autoimmune Disease.
    J Immunother 2016 May;39(4):188-90
    Department of Dermatology, University Hospital Schleswig-Holstein (UKSH), Campus Kiel, Kiel, Germany.
    Pembrolizumab, an anti-programmed death-1 monoclonal antibody, has been approved by the Food and Drug Administration in 2014 on the basis of improved progression-free and overall survival in metastatic melanoma. We report for the first time a successful treatment with a programmed death-1 antibody in a 69-year-old metastastic melanoma patient with a Churg-Strauss lung vasculitis and a prior ipilimumab-induced autoimmune colitis. This case report suggests that pembrolizumab can be given with caution to patients with underlying autoimmune disease. Read More

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