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    2622 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis

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    Innate immune response reflects disease activity in eosinophilic granulomatosis with polyangiitis.
    Clin Exp Allergy 2018 Jun 16. Epub 2018 Jun 16.
    Clinical Research Center, National Hospital Organization Sagamihara National Hospital, 18-1 Sakuradai, Minami-ku Sagamihara, Kanagawa, Japan, 252-0392.
    Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a disease characterized by allergic granulomatosis, necrotizing vasculitis, and peripheral blood eosinophilia. Interleukin (IL)-33, thymic stromal lymphopoietin (TSLP), and type-2 innate lymphoid cells (ILC2) are involved in the innate and type-2 immune responses in EGPA. However, the relationships among these molecules and the mechanisms underlying the development of EGPA remain unknown. Read More

    Eosinophilic Granulomatosis with Polyangiitis Manifested by Cholecystitis and Mononeuritis Multiplex: A Case Report.
    Iran J Med Sci 2018 May;43(3):332-335
    Resident in Internal Medicine, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.
    Eosinophilic granulomatosis with polyangiitis formerly named "Churg-Strauss syndrome (CSS)" is a systemic disease with bronchial asthma, hypereosinophilia, and systemic vasculitis. We report a case of CSS with cholecystitis and mononeuritis multiplex. A 50-year-old woman with a history of sinusitis and bronchial asthma of 8 years' duration was referred with a complaint of left-hand deformity and difficulty in walking. Read More

    Eosinophilic granulomatosis with polyangiitis and laryngeal involvement: review of the literature and a cross-sectional prospective experience.
    J Laryngol Otol 2018 Jun 11:1-5. Epub 2018 Jun 11.
    Rheumatology Unit,University of Pisa,Italy.
    Background: Eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis show variable otorhinolaryngological involvement. Up to 14 per cent of granulomatosis with polyangiitis patients have subglottis involvement; little is known about the laryngeal involvement in eosinophilic granulomatosis with polyangiitis.

    Method: A literature review was conducted, together with a prospective cross-sectional analysis of 43 eosinophilic granulomatosis with polyangiitis patients. Read More

    Cardiogenic Shock: An Unusual Initial Presentation of Churg-Strauss Syndrome.
    Case Rep Rheumatol 2018 1;2018:2076837. Epub 2018 Apr 1.
    Teaching Hospital Jaffna, Faculty of Medicine, University of Jaffna, Jaffna, Sri Lanka.
    Churg-Strauss syndrome (CSS) is a rare autoimmune condition, characterized by necrotizing extravascular eosinophil rich granulomatous inflammation of the tissues and disseminated small-medium sized vessel vasculitis in a patient with bronchial asthma and tissue eosinophilia. Though pulmonary involvement is the predominant feature of CSS, extra pulmonary involvement, in particular, cardiac involvement, denotes an adverse outcome. Here we report a 50-year-old female who presented with cardiogenic shock due to an acute coronary event as the initial manifestation of CSS. Read More

    Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review.
    Drugs 2018 May 15. Epub 2018 May 15.
    Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Université Paris Descartes, Paris, France.
    Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis. Nevertheless, eosinophilic granulomatosis with polyangiitis stands apart because it has features of vasculitis and eosinophilic disorders that require targeted therapies somewhat different from those used for other anti-neutrophil cytoplasm antibody-associated vasculitides. Considerable advances have been made in understanding the underlying pathophysiology of eosinophilic granulomatosis with polyangiitis that have highlighted the key role of eosinophils and opened new therapeutic opportunities. Read More

    [Clinical Analysis of 40 Patients with Eosinophilic Lung Diseases in Peking Union Medical College Hospital].
    Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2018 Apr;40(2):170-177
    Department of Respiratory Medicine,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
    Objective To summarize the clinical features of eosinophilic lung diseases(ELD). Methods We retrospectively analyzed the clinical manifestations,laboratory findings,accessory examination results,and pathology of 40 patients who were diagnosed with ELD and hospitalized in Peking Union Medical College Hospital from January 2013 to December 2016.Results There were 19 males and 21 females,and the average age was(48. Read More

    A 92-Year-Old Male with Eosinophilic Asthma Presenting with Recurrent Palpable Purpuric Plaques.
    Dermatopathology (Basel) 2018 Jan-Mar;5(1):44-48. Epub 2018 Mar 16.
    Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA.
    Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis affecting the small and medium-sized vasculature. It is commonly associated with asthma and eosinophilia. Most patients are diagnosed at around the age of 40. Read More

    Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis.
    Drugs Today (Barc) 2018 Feb;54(2):93-101
    Royal Brompton Hospital, London, UK.
    Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare but potentially life-threatening antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis which affects, to varying degrees, the lungs, paranasal sinuses, heart, kidneys, skin and peripheral nervous system. It is strongly associated with asthma. Peripheral eosinophilia is a defining feature of EGPA and eosinophilic inflammation is often observed in biopsies of affected tissues. Read More

    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 Jun 21;37(6):1729-1735. 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

    Etiology of cutaneous vasculitis: utility of a systemic approach
    Gac Med Mex 2018 ;154(1):62-67
    Subdirección de Investigación Biomédica, Hospital General Dr. Manuel Gea González, Ciudad de México, México.
    Cutaneous vasculities (CV) represents a diagnostic challenge, occurs as primary cutaneous disorder or as a manifestation of other entities.

    Objective: To search the cause of CV. Methods: Patients with CV were prospectively evaluated. 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 Jun 5;18(6):1544-1547. Epub 2018 Mar 5.
    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

    CHARACTERISTICS OF ARTICULAR SYNDROME IN SYSTEMIC VASCULITIS.
    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) 2018 May;59(5):497-504
    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

    Adverse drug reactions of montelukast in children and adults.
    Pharmacol Res Perspect 2017 10;5(5)
    Department of Pediatrics, Medical Center Leeuwarden, Leeuwarden, The Netherlands.
    Montelukast, a selective leukotriene receptor antagonist, is recommended in guidelines for the treatment of asthma in both children and adults. However, its effectiveness is debated, and recent studies have reported several adverse events such as neuropsychiatric disorders and allergic granulomatous angiitis. This study aims to obtain more insight into the safety profile of montelukast and to provide prescribing physicians with an overview of relevant adverse drug reactions in both children and adults. Read More

    Successful treatment using rituximab in a patient with refractory polymyositis complicated by scleroderma renal crisis.
    BMJ Case Rep 2017 Sep 23;2017. Epub 2017 Sep 23.
    Department of Rheumatology, Kawasaki Medical School, Kurashiki, Japan.
    Corticosteroids are the first-line treatment for patients with inflammatory myopathies. Myositis can be a clinical feature of scleroderma (polymyositis-scleroderma overlap syndrome), and treatment of this syndrome is a challenge for clinicians because moderate to high doses of corticosteroids are considered a risk factor for development of acute kidney injury in affected patients. We report here the case of a 56-year-old woman with scleroderma who developed polymyositis and was successfully treated with rituximab. 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

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