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    2558 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis

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    Neurological Complications in Eosinophilic Granulomatosis with Polyangiitis (EGPA): The Roles of History and the Results of Physical Examinations in the Diagnosis of EGPA.
    Intern Med 2017 Sep 15. 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 Sep 12. 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

    ANCA Glomerulonephritis and Vasculitis.
    Clin J Am Soc Nephrol 2017 Aug 25. 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 Jul 31. 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

    [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 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

    Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis.
    Clin Exp Rheumatol 2017 Mar-Apr;35 Suppl 103(1):111-118. Epub 2017 Mar 23.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
    Objectives: We reclassified Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) into 3 categories of AAV including MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis, and investigated clinical and prognostic features.

    Methods: We reviewed the medical records of 133 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), who had either myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or no ANCA, and who had ever achieved the first remission. We compared clinical manifestations, initial Birmingham vasculitis activity score (BVAS) and five factor score (FFS), and relapse rates. Read More

    Cardiac involvement as a presenting feature of eosinophilic granulomatosis with polyangiitis in childhood.
    Clin Exp Rheumatol 2017 Mar-Apr;35 Suppl 103(1):225. Epub 2017 Mar 15.
    Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol & Royal National Hospital for Rheumatic Diseases NHS Foundation Trust, Bath, UK.
    ****************************************************************************. 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 Jul;40(7):559-570
    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

    IgA antibodies to myeloperoxidase in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
    Clin Exp Rheumatol 2017 Mar-Apr;35 Suppl 103(1):98-101. Epub 2017 Mar 1.
    Section of Rheumatology, Boston University School of Medicine; and Rheumatology Section, VA Boston Healthcare System, Boston, MA, USA.
    Objectives: To determine the prevalence of anti-myeloperoxidase (MPO) antibodies of IgA (IgA anti-MPO) isotype in patients with eosinophilic granulomatosis with polyangiitis (EGPA), and the association of the IgA antibodies with IgG anti-MPO and with disease activity.

    Methods: Serum samples from patients with EGPA followed in a multicenter longitudinal cohort were tested by ELISA for the presence of IgA anti-MPO and IgG anti-MPO antibodies. Sera from 87 healthy controls were used to define a positive test. Read More

    Hospital Morbidity Database for Epidemiological Studies on Churg-Strauss Syndrome.
    Adv Exp Med Biol 2017 ;980:19-25
    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

    Neutrophil extracellular traps formation in patients with eosinophilic granulomatosis with polyangiitis: association with eosinophilic inflammation.
    Clin Exp Rheumatol 2017 Mar-Apr;35 Suppl 103(1):27-32. Epub 2017 Feb 9.
    Institute of Cardiology, Jagiellonian University Medical College, Krakow; and John Paul II Hospital, Kraków, Poland.
    Objectives: Eosinophilic granulomatosis with polyangiitis (EGPA) is associated with an inflammation and the presence of antineutrophil cytoplasmic antibodies (ANCA). Thus, we investigated the impact of ANCAs and eosinophilic inflammation on neutrophil activation and extracellular traps (NETs) formation.

    Methods: We recruited 29 patients in the remission of EGPA (17 ANCA-negative and 12 ANCA-positive, including 7 p-ANCA-positive and 5 c-ANCA-positive patients). 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 Jun 11;42(6):478.e1-478.e4. 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

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