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    2604 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis

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    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

    [Manifestation of eosinophilic granulomatosis with polyangiitis in the head and neck area over time taking systemic disease activity into consideration].
    Z Rheumatol 2018 Mar 22. Epub 2018 Mar 22.
    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

    Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report.
    Clin Rheumatol 2018 Mar 21. Epub 2018 Mar 21.
    Department of Vascular Problems in Rheumatic Diseases, V.A. Nasonova Research Institute of Rheumatology, Kashirskoe shosse 34A, Moscow, Russian Federation, 115522.
    Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Read More

    Churg-Strauss syndrome with cardiac involvement: case illustration and contribution of CMR in the diagnosis and clinical follow-up.
    Int J Cardiol 2018 May 3;258:321-324. Epub 2018 Feb 3.
    Service de cardiologie, Centre Hospitalier Universitaire Vaudois, Switzerland.
    This report summarises three cases of Churg-Strauss syndrome (CSS) illustrating the diagnostic challenges associated with the cardiac manifestation of this disease. Here, we illustrate the role of cardiac magnetic resonance (CMR) for diagnosis and follow-up of CSS with a focus on new non-contrast T-weighted imaging sequences for quantification of myocardial scar tissue and quantitative T mapping techniques, which allow the detection of myocardial edema. Read More

    Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients.
    Turk Thorac J 2017 Jul 1;18(3):72-77. Epub 2017 Jul 1.
    Department of Chest Diseases, Erciyes University School of Medicine, Kayseri, Turkey.
    Objectives: Eosinophilic granulomatosis with poliangiitis (EGPA) which was previously called Churg-Strauss Syndrome, is classified into eosinophilic and vasculitic phases. To characterize the eosinophilic and vasculitic phases of the disease in terms of clinical findings, serology, and treatment.

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

    Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature.
    Int Arch Allergy Immunol 2018 23;175(1-2):61-69. Epub 2018 Jan 23.
    Respiratory Pathophysiology Unit, Cardiothoracic and Vascular Department, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.
    Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. Read More

    Recurrence of eosinophilic granulomatosis with polyangitis after orthotopic heart transplant.
    Am J Transplant 2018 Feb 2. Epub 2018 Feb 2.
    Department of Cardiology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
    Eosinophilic granulomatosis with polyangitis (EGPA), previously referred to as Churg-Strauss syndrome, is a necrotizing small vessel vasculitis associated with eosinophilic infiltrates and extravascular granulomas. We report a case of a Caucasian woman successfully bridged to heart transplantation with a continuous flow left ventricular assist device (LVAD) who survived recurrence of EGPA in the allograft. Read More

    Georgian Med News 2017 Dec(273):69-75
    Donetsk National Medical University of Health Ministry of Ukraine, Lyman; State Institution "Dnipropetrovsk Medical Academy of the Ministry of Health of Ukraine", Ukraine.
    The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1. Joint's damage in the form of arthritis or arthralgia observed in 32-67% different groups of patients, that depending on the disease duration, the degree of the pathological process's activity, extraarticular signs severity, lung parenchyma involving and hemodynamic status in the pulmonary circulation. Read More

    Polyps, grommets and eosinophilic granulomatosis with polyangiitis.
    J Laryngol Otol 2018 Mar 9;132(3):236-239. Epub 2018 Jan 9.
    Department of Otolaryngology, Head and Neck Surgery,University Hospital Limerick,Ireland.
    Objective: To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.

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

    Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Complicated by Perforation of the Small Intestine and Cholecystitis.
    Intern Med 2018 Mar 21;57(5):737-740. Epub 2017 Dec 21.
    Division of Neurology, Department of Internal Medicine, Tokai University School of Medicine, Japan.
    We report a case of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) complicated by perforation of the small intestine and necrotizing cholecystitis. A 69-year-old man with a history of bronchial asthma was admitted with mononeuritis multiplex. The laboratory findings included remarkable eosinophilia. Read More

    Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis.
    An Bras Dermatol 2017 ;92(5 Suppl 1):56-58
    Dermatology Outpatient Clinic, Universidade de Mogi das Cruzes - Mogi das Cruzes (SP), Brazil.
    Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions. Read More

    Wells syndrome secondary to influenza vaccination: A case report and review of the literature.
    Hum Vaccin Immunother 2018 Apr 18;14(4):958-960. Epub 2018 Jan 18.
    b Division of Internal Medicine, McGill University Health Center , Montreal , Quebec , Canada.
    Vaccinations have been shown repeatedly to be extremely safe with low incidence of complications. Given the rarity, these adverse events, they must be reported and examined cautiously. This case report illustrates the first case of an adult presenting with Wells syndrome that developed soon after vaccination with the thiomersal, a common preservative, containing influenza vaccine. Read More

    Churg-strauss syndrome: a case report.
    Wiad Lek 2017;70(5):992-994
    Higher State Educational Establishment Of Ukraine "Ukrainian Medical Stomatological Academy", Poltava, Ukraine.
    A clinical case of Churg-Strauss syndrome has been reported on the 53-year-old female patient Ts. with bronchial asthma and allergic rhinitis. The main clinical signs and syndromes depending on the stage of the disease are presented, as well as therapeutic treatment of patients with this disease. Read More

    Eosinophilic granulomatosis with polyangiitis without respiratory symptoms or asthma in an adolescent: case report and literature review.
    Rheumatol Int 2018 Apr 30;38(4):697-703. Epub 2017 Nov 30.
    Department of Pediatric Rheumatology, Pamukkale University School of Medicine, Pamukkale, Denizli, Turkey.
    Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. Read More

    Eosinophilic Myocarditis.
    Am J Med Sci 2017 11 6;354(5):486-492. Epub 2017 Apr 6.
    Division of Hematology, University of British Columbia, Vancouver, British Columbia. Electronic address:
    Persistent eosinophilia can cause cardiac tissue damage, typically in the form of eosinophilic myocarditis, whether the underlying cause is reactive, a clonal myeloid disorder, or idiopathic hypereosinophilic syndrome (HES). Eosinophilic myocarditis ranges from mild localized disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications and endomyocardial fibrosis. Systemic treatment varies widely depending on the underlying cause, so thorough investigation and precise diagnosis are essential. Read More

    Eosinophilic myocarditis as a first presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
    BMJ Case Rep 2017 Nov 23;2017. Epub 2017 Nov 23.
    Department of Cardiology, Mercy University Hospital, Cork, Ireland.
    We present the case of a 28-year-old man who presented with chest pain and elevated cardiac biomarkers, with no evidence of acute ischaemia. He had a pronounced eosinophilia, abnormal echocardiographic, cardiac MRI and CT findings. He underwent transbronchial biopsy of carinal lymph nodes and of lung parenchyma. Read More

    Involvement of the Peripheral Nervous System in Polyarteritis Nodosa and Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis.
    Rheum Dis Clin North Am 2017 Nov 19;43(4):633-639. Epub 2017 Aug 19.
    Department of Medicine, University of California, Box 0868, San Francisco, CA 94143, USA. Electronic address:
    Peripheral nerve involvement is common in polyarteritis nodosa and the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. The underlying mechanism is arteritis of the vasa nervorum, leading to ischemic neuropathy. The classic presentation is stepwise involvement of peripheral nerves with ongoing antecedent constitutional symptoms. Read More

    Central Nervous System Disease in Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis.
    Rheum Dis Clin North Am 2017 Nov;43(4):573-578
    Division of Rheumatology, University of California, Zuckerberg San Francisco General Hospital, San Francisco, 1001 Potrero Avenue, Box 0868, San Francisco, CA 94143, USA. Electronic address:
    Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease. Read More

    A case of fatal perimyocarditis due to a rare disease.
    Forensic Sci Med Pathol 2017 Dec 11;13(4):454-458. Epub 2017 Oct 11.
    Institute of Legal Medicine, University Hospital Frankfurt, Kennedyallee 104, 60596, Frankfurt am Main, Germany.
    Although myocarditis is caused by viral infections in about 50% of cases in European countries, various other causative agents are known. We report the case of a 51-year-old man who died several months after being diagnosed with asthma by his general practitioner. This diagnosis had been confirmed by a pulmonologist approximately 6 weeks before the man's death. Read More

    Methotrexate versus cyclophosphamide for remission maintenance in ANCA-associated vasculitis: A randomised trial.
    PLoS One 2017 10;12(10):e0185880. Epub 2017 Oct 10.
    Nephrology Unit, University Hospital of Parma, Italy.
    Objectives: The treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is based on remission-induction and remission-maintenance. Methotrexate is a widely used immunosuppressant but only a few studies explored its role for maintenance in AAV. This trial investigated the efficacy and safety of methotrexate as maintenance therapy for AAV. Read More

    [Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].
    Internist (Berl) 2017 Oct 5. Epub 2017 Oct 5.
    Klinik für Innere Medizin, Kreiskrankenhaus Frankenberg, Frankenberg (Eder), Deutschland.
    This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). Read More

    An atypical presentation of cardiac tamponade and periorbital swelling in a patient with eosinophilic granulomatosis with polyangiitis: a case report.
    J Med Case Rep 2017 Sep 24;11(1):271. Epub 2017 Sep 24.
    Department of Medicine, University of Utah School of Medicine, Salt Lake City, UT, USA.
    Background: Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated.

    Case Presentation: We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. Read More

    Neurological Complications in Eosinophilic Granulomatosis with Polyangiitis (EGPA): The Roles of History and Physical Examinations in the Diagnosis of EGPA.
    Intern Med 2017 Nov 15;56(22):3003-3008. Epub 2017 Sep 15.
    Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Japan.
    Objective To investigate the clinical symptoms, the physical and neurological findings, and the clinical course of neurological complications in eosinophilic granulomatosis with polyangiitis (EGPA). Methods A retrospective chart review of EGPA cases managed by two referral hospitals was performed, with a focus on the neurological findings. The study analyzed the symptoms at the onset of EGPA and investigated their chronological relationship. Read More

    Rituximab as Induction Therapy in Eosinophilic Granulomatosis with Polyangiitis Refractory to Conventional Immunosuppressive Treatment: A 36-Month Follow-Up Analysis.
    J Allergy Clin Immunol Pract 2017 Nov - Dec;5(6):1556-1563. Epub 2017 Sep 12.
    Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. Electronic address:
    Background: Rituximab (RTX) is approved for induction therapy of granulomatosis with polyangiitis and microscopic polyangiitis. In eosinophilic granulomatosis with polyangiitis (EGPA), organ-threatening manifestations are mainly treated with cyclophosphamide (CYC). RTX as treatment in EGPA has been described in small case series; however long-term data and the efficacy of RTX in EGPA refractory to CYC have not been reported yet. Read More

    The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study.
    Arthritis Rheumatol 2017 12 9;69(12):2338-2350. Epub 2017 Nov 9.
    Mayo Clinic College of Medicine and Science, Rochester, Minnesota.
    Objective: To estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), in a US-based adult population.

    Methods: All medical records of patients with a diagnosis of, or suspicion of having, AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. AAV incidence rates were age- and sex-adjusted to the 2010 US white population. Read More

    ANCA Glomerulonephritis and Vasculitis.
    Clin J Am Soc Nephrol 2017 Oct 25;12(10):1680-1691. Epub 2017 Aug 25.
    Department of Pathology and Laboratory Medicine, Department of Medicine, and Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
    ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). Read More

    Peripheral Ulcerative Keratitis Associated with Autoimmune Disease: Pathogenesis and Treatment.
    J Ophthalmol 2017 13;2017:7298026. Epub 2017 Jul 13.
    Department of Ophthalmology, China-Japan Union Hospital of Jilin University, Changchun City, China.
    Peripheral ulcerative keratitis (PUK) is type of crescent-shaped inflammatory damage that occurs in the limbal region of the cornea. PUK is always combined with an epithelial defect and the destruction of the peripheral corneal stroma. PUK may have a connection to systemic conditions, such as long-standing rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Wegener granulomatosis (WG), relapsing polychondritis, classic polyarteritis nodosa and its variants, microscopic polyangiitis, and Churg-Strauss syndrome. Read More

    Sequential rituximab and omalizumab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
    Clin Rheumatol 2017 Sep 31;36(9):2159-2162. Epub 2017 Jul 31.
    GIRAT: Grupo de Investigación en Reumatología, Autoinmunidad y Medicina Traslacional, Fundación Valle del Lili and Univesidad Icesi, Cali, Colombia.
    Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Read More

    Diagnosis of Churg-Strauss Syndrome Presented With Neuroendocrine Carcinoma: A Case Report.
    Ann Rehabil Med 2017 Jun 29;41(3):493-497. Epub 2017 Jun 29.
    Department of Physical Medicine and Rehabilitation, Dongguk University College of Medicine, Seoul, Korea.
    Churg-Strauss syndrome (CSS) is a rare systemic vasculitis that affect small and medium-sized blood vessels and is accompanied by asthma, eosinophilia, and peripheral neuropathy. This report describes a case of a 52-year-old man who had a history of sinusitis, asthma, and thymus cancer and who had complained of bilateral lower extremity paresthesia and weakness for a month. Peripheral neuropathy was detected by electrodiagnostic studies. Read More

    Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis.
    Exp Neurobiol 2017 Jun 14;26(3):168-171. Epub 2017 Jun 14.
    Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul 06973, Korea.
    Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). Read More

    Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors: A Randomized, Controlled Trial.
    Arthritis Rheumatol 2017 11 15;69(11):2175-2186. Epub 2017 Oct 15.
    National Referral Center for Rare Systemic and Autoimmune Diseases, Department of Internal Medicine, Université Paris Descartes, Hôpital Cochin, AP-HP, Paris, France.
    Objective: In most patients with nonsevere systemic necrotizing vasculitides (SNVs), remission is achieved with glucocorticoids alone, but one-third experience a relapse within 2 years. This study was undertaken to determine whether the addition of azathioprine (AZA) to glucocorticoids could achieve a higher sustained remission rate of newly diagnosed nonsevere eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), microscopic polyangiitis (MPA), or polyarteritis nodosa (PAN).

    Methods: All patients included in this double-blind trial received glucocorticoids, gradually tapered over 12 months, and were randomized to receive AZA or placebo for 12 months, with stratification according to SNV (EGPA or MPA/PAN). Read More

    [ANCA-associated vasculitides at Mexico City's metropolitan Eastern area].
    Rev Med Inst Mex Seguro Soc 2017 Jul-Aug;55(4):430-440
    Consulta Externa de Reumatología, Hospital General Regional 25, Instituto Mexicano del Seguro Social, Ciudad de México, México.
    Background: The anti-neutrophil cytoplasmic antibody-associated (ANCA) vasculitides includes granulomatosis with polyangiitis (Wegener's) (GPW), Chrug-Strauss syndrome (CSS) and microscopic polyangiitis (MPA). Since it has low incidence in our field, there are a few published papers. The aim of this study was to report the clinical characteristics, activity and damage of these vasculitides in Mexico City's metropolitan eastern area. Read More

    Cardiac involvements in hypereosinophilia-associated syndrome: Case reports and a little review of the literature.
    Echocardiography 2017 Aug 1;34(8):1242-1246. Epub 2017 Jun 1.
    The Department of Cardiovascular Ultrasound, The First Hospital of China Medical University, Shenyang, Liaoning, China.
    Hypereosinophilia-associated syndrome is a rare group of systemic diseases without certain underlying causes. Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), are initial considerations, when underlying causes remains unexplained despite of complete evaluation of hypereosinophilia. In this study, we report two rare cases, one case of HES with Loeffler endocarditis, and the other one of EGPA with restrictive cardiomyopathy mimicking myocardial infarction, to further address differential chief cardiac manifestations between HES and EGPA. Read More

    Longterm Prognosis of 121 Patients with Eosinophilic Granulomatosis with Polyangiitis in Japan.
    J Rheumatol 2017 Aug 1;44(8):1206-1215. Epub 2017 Jun 1.
    From the Department of Respirology, National Hospital Organization Saitama National Hospital, Saitama; Department of Allergy and Respirology, National Hospital Organization Sagamihara National Hospital, Minami-ku Sagamihara, Japan.
    Objective: We investigated the risk factors for relapse or prognosis of eosinophilic granulomatosis with polyangiitis (EGPA) in Japanese patients presenting to our hospital.

    Methods: From June 1999 through March 2015, we retrospectively recruited 121 patients with EGPA according to the American College of Rheumatology criteria. Frequent relapse was defined as disease occurrence at least once every 2 years after a period of initial remission. Read More

    Eosinophilic granulomatosis with polyangiitis and mononeuritis multiplex responded to induction cyclophosphamide.
    BMJ Case Rep 2017 May 27;2017. Epub 2017 May 27.
    Internal Medicine, Health Campus, University Sains Malaysia, Kota Bharu, Kelantan, Malaysia.
    We report a case of 28-year-old Malay woman who initially presented with multiple joints pain with underlying poorly controlled asthma since her childhood. She was treated as seronegative arthritis. However, the involvement of joints, asthma and high-eosinophil counts raised suspicion of Churg-Strauss syndrome. Read More

    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

    Interferon-α for Induction and Maintenance of Remission in Eosinophilic Granulomatosis with Polyangiitis: A Single-center Retrospective Observational Cohort Study.
    J Rheumatol 2017 Jun 15;44(6):806-814. Epub 2017 Apr 15.
    From the Department of Respiratory Medicine, Hannover Medical School, Hannover; Medical Clinic III, University Clinic Jena, Jena, Germany; Kantonsspital St. Gallen, St. Gallen, Switzerland.
    Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by frequent relapses following induction therapy. Interferon-α (IFN-α) can reverse the underlying Th2-driven immune response and has successfully induced remission in previous reports. We undertook this study to investigate its efficacy and safety in patients with EGPA. Read More

    Are the 1990 American College of Rheumatology vasculitis classification criteria still valid?
    Rheumatology (Oxford) 2017 Jul;56(7):1154-1161
    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.

    Methods: The Diagnosis and Classification in Vasculitis Study provided detailed clinical, serological, pathological and radiological data from patients with primary systemic vasculitis and clinical context-specific comparator conditions. Read More

    Future Prospects of Biologic Therapies for Immunologic Diseases.
    Immunol Allergy Clin North Am 2017 05;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

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