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    [Extranodal NK-T-cell lymphoma, nasal type in granulomatosis with polyangiitis. A case report].
    Rev Med Inst Mex Seguro Soc 2017 May-Jun;55(3):394-398
    Departamento de Medicina Interna, Hospital de Especialidades "Antonio Fraga Mouret", Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Ciudad de México, México.
    Background: Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes.

    Clinical Case: Male patient admitted due to an unexplained fever and weight loss; it was diagnosed GP by a history of pauci-immune glomerulonephritis, fixed pulmonary nodules and chronic sinusitis of four years of evolution; however, the fever persisted despite treatment and in the absence of infection. Read More

    [The first clinical description of granulomatosis with polyangiitis (known before as Wegener's granulomatosis)].
    Rev Med Inst Mex Seguro Soc 2017 May-Jun;55(3):380-381
    Hospital General Mexicali, Instituto de Servicios de Salud Pública del Estado de Baja California, Secretaría de Salud, Mexicali, Baja California, México.
    Before 1950, cases of necrotizing vasculitis were commonly published in journals of pathology. Most of these cases were designated as polyarteritis nodosa. In 1952, the pathologist Pearl Zeek critically reviewed and summarized the literature dealing with polyarteritis nodosa and first grouped the different types of necrotizing vasculitis. Read More

    The Successful Treatment of Myeloperoxidase Antineutrophil Cytoplasmic Antibody-positive Hypertrophic Pachymeningitis in Patients with the Limited Form of Granulomatosis with Polyangiitis Using Methotrexate: Two Case Reports.
    Intern Med 2017 15;56(8):959-965. Epub 2017 Apr 15.
    Department of Medicine, Division of Respirology, Neurology and Rheumatology, Kurume University School of Medicine, Japan.
    Recent findings have indicated a close relationship between myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive hypertrophic pachymeningitis and the limited form of granulomatosis with polyangiitis (GPA). In Japan, MPO-ANCA-positive hypertrophic pachymeningitis predominantly occurs in elderly individuals. We herein describe the cases of two patients with MPO-ANCA-positive hypertrophic pachymeningitis associated with the limited form of GPA who were successfully treated with a combination of corticosteroids and methotrexate. Read More

    Pulmonary Perfusion Scan Mimicking Hepatobiliary Scintigraphy in a Patient With an Uncommon Manifestation of Granulomatosis With Polyangiitis.
    Clin Nucl Med 2017 Apr 17. Epub 2017 Apr 17.
    From the Department of Nuclear Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA.
    A 36-year-old man with known granulomatosis with polyangiitis underwent quantitative ventilation-perfusion pulmonary scintigraphy as part of an initial evaluation for lung transplant candidacy. Radiotracer uptake on the perfusion study has the appearance of the hepatic silhouette, drawing initial concern that there may have been a misadministration of Tc with a hepatobiliary agent. Comparison CT reveals extensive cystic bronchiectasis, parenchymal destruction, and volume loss involving the entire left lung and basal portions of the right lung, with the residual portions of functional lung parenchyma conforming to a shape similar to the liver. Read More

    Semaphorin 4D inhibits neutrophil activation and is involved in the pathogenesis of neutrophil-mediated autoimmune vasculitis.
    Ann Rheum Dis 2017 Apr 17. Epub 2017 Apr 17.
    Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, Suita City, Osaka, Japan
    Objectives: Inappropriate activation of neutrophils plays a pathological role in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The aim of this study was to investigate the functions of semaphorin 4D (SEMA4D) in regulation of neutrophil activation, and its involvement in AAV pathogenesis.

    Methods: Serum levels of soluble SEMA4D were evaluated by ELISA. Read More

    Additional Risk Minimisation Measures for Medicinal Products in the European Union: A Review of the Implementation and Effectiveness of Measures in the United Kingdom by One Marketing Authorisation Holder.
    Pharmaceut Med 2017 29;31(2):101-112. Epub 2017 Mar 29.
    School of Life and Medical Sciences, University of Hertfordshire, Hertfordshire, AL10 9AB UK.
    Introduction: Additional risk minimisation measures (aRMMs) for medicinal products are necessary to address specific important safety issues which may not be practically achieved through routine risk management measures alone. The implementation and determination of effectiveness for aRMMs can be a challenge as it involves multiple stakeholders. It is therefore important to have concise objectives to avoid undue burden on patients, healthcare professionals and the healthcare system. Read More

    [Pachymeningitis].
    Rev Med Interne 2017 Apr 13. Epub 2017 Apr 13.
    Service de médecine interne, hôpital Saint-Antoine, AP-HP, DHUi2B, université Paris 6, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.
    Pachymeningitis is a fibrosing and inflammatory process, which involves the dura mater. Some pachymeningitis are cranial and induce headaches and cranial nerve palsies. Others are spinal and responsible for nerve roots or spinal cord compression. 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 Apr 15. 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. B. Seeliger, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, and Department of Respiratory Medicine, Hannover Medical School; M. Förster, PhD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; J. Happe, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; T. Forberg, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; A. Moeser, MD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital; T. Neumann, MD, Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, and Department of Rheumatology, Kantonsspital St. Gallen; C. Kroegel, MD, PhD, Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital. Dr. Neumann and Dr. Kroegel contributed equally to this manuscript. Address correspondence to Dr. B. Seeliger, Hannover Medical School, Department of Respiratory Medicine, Carl-Neuberg-Str. 1, 30625 Hannover, Germany. E-mail: Accepted for publication January 19, 2017.
    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

    Lung involvement in childhood onset granulomatosis with polyangiitis.
    Pediatr Rheumatol Online J 2017 Apr 14;15(1):28. Epub 2017 Apr 14.
    Pediatric Clinic, Department of Surgical and Biomedical Sciences, Università degli Studi di Perugia, Perugia, Italy.
    Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Read More

    [Varicella gastritis under immunosuppression : Case report of a woman after lung transplantation due to granulomatosis with polyangiitis].
    Internist (Berl) 2017 Apr 12. Epub 2017 Apr 12.
    Abteilung Innere Medizin II (Onkologie, Hämatologie, Rheumatologie, Immunologie, Pulmonologie), Universitätsklinikum Tübingen, Otfried-Müller-Str. 10, 72076, Tübingen, Deutschland.
    A 35-year-old woman who had previously undergone a lung transplantation presented with severe abdominal pain and vomiting. The gastroscopy showed diffuse ulcerative gastric lesions. Tests for varicella zoster virus and Epstein-Barr virus via polymerase chain reactions (PCR) on endoscopically obtained gastric biopsies were found to be positive and confirmed varicella gastritis. Read More

    Renal allograft granulomatous interstitial nephritis: observations of an uncommon injury pattern in 22 transplant recipients.
    Clin Kidney J 2017 Apr 1;10(2):240-248. Epub 2017 Feb 1.
    University of Chicago, Chicago, IL USA; Sharp Memorial Hospital, San Diego, CA, USA.
    Background: Granulomatous interstitial nephritis (GIN) is uncommon in native kidneys, and descriptions in allografts are few. We report clinical and pathologic findings in 22 allograft recipients with GIN identified in renal allograft biopsies and nephrectomies. Methods: Renal allografts with GIN were retrieved from the pathology files of two academic medical centers. Read More

    Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up.
    Tuberk Toraks 2016 Sep;64(3):223-229
    Department of Chest Diseases, Baskent University Adana Dr. Turgut Noyan Teaching and Medical Research Center, Adana, Turkey.
    Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital.

    Patients And Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014.

    Result: The mean time from the onset of symptoms to diagnosis was 7. Read More

    Th17 and Treg lymphocytes as cellular biomarkers of disease activity in Granulomatosis with Polyangiitis.
    Eur J Immunol 2017 Apr;47(4):633-636
    Department of Experimental and Clinical Medicine and DENOTHE Center, University of Florence, Florence, Italy.
    Granulomatosis with Polyangiitis (GPA) (formerly known as Wegener's granulomatosis) is a vasculitis of unknown etiology affecting predominantly small- to medium-sized vessels, usually involving the upper and lower respiratory tract and kidneys. Anti-neutrophil cytoplasmic autoantibodies are probably the initial cause of the inflammatory process that leads to the typical necrotizing lesions. In this issue of the European Journal of Immunology, Szczeklik et al. Read More

    An Unusual Presentation of Limited Granulomatosis with Polyangiitis Involving Vagina and Urethra.
    Case Rep Rheumatol 2017 13;2017:9407675. Epub 2017 Mar 13.
    Department of Urology, Complejo Hospitalario Universitario de Albacete, Albacete, Spain.
    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatosis vasculitis characterized by predilection to affect small- and medium-sized blood vessels and commonly affects the upper and lower respiratory tract and kidneys in most cases. Genital involvement is reported in <1% of cases in large cohorts and nearly all cases have been in the setting of multisystemic disease or during the course of the disease in patients already diagnosed as GPA. A case is presented of uncommon limited urogenital GPA in a 66-year-old woman with an irregular mass occupying urethra and vagina. Read More

    Patients with ANCA-associated vasculitis admitted to the intensive care unit with acute vasculitis manifestations: a retrospective and comparative multicentric study.
    Ann Intensive Care 2017 Dec 5;7(1):39. Epub 2017 Apr 5.
    Néphrologie-Dialyse-Transplantation, CHU Angers, 4 rue Larrey, 49933, Angers Cedex 9, France.
    Purpose: Data for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce.

    Methods: We included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002-2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group. Read More

    Autoimmune-related nasal septum perforation: A case report and systematic review.
    Allergy Rhinol (Providence) 2017 Mar;8(1):40-44
    Background: Inflammatory injury of nasal respiratory mucosa is a common feature of multisystem autoimmune disease. Certain autoimmune disorders are associated with nasal septum perforation (NSP). We performed a systematic review of the literature to better understand the association of NSP with specific autoimmune disorders. Read More

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

    Spectrum of immune-related conditions associated with risk of keratinocyte cancers among elderly adults in the United States.
    Cancer Epidemiol Biomarkers Prev 2017 Apr 4. Epub 2017 Apr 4.
    Division of Cancer Epidemiology and Genetics, National Cancer Institute.
    Elevated keratinocyte carcinoma (KC) risk is present with several immune-related conditions, e.g., solid organ transplantation and non-Hodgkin lymphoma. Read More

    Pachymeningitis and cerebral granuloma in granulomatosis with polyangiitis: is rituximab a promising treatment option?
    Acta Reumatol Port 2017 Jan-Mar;42(1):82-87
    Centro Hospitalar e Universitário de Coimbra.
    Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease characterized by granulomatous inflammation involving upper and lower respiratory tract, kidneys and peripheral nervous system. However, central nervous system involvement is uncommon and frequently refractory to classical therapy. Rituximab has emerged as promising alternative, but published reports are scarce. Read More

    [Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up].
    Tuberk Toraks 2016 Sep;64(3):223-229
    Department of Chest Diseases, Baskent University Adana Dr. Turgut Noyan Teaching and Medical Research Center, Adana, Turkey.
    Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital.

    Patients And Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014.

    Result: The mean time from the onset of symptoms to diagnosis was 7. 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

    ANCA-Associated Vasculitis Pathogenesis: A Commentary.
    Curr Rheumatol Rep 2017 Apr;19(4):15
    Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, MFL Center Tower, Ste. 5300, Baltimore, MD, 21224, USA.
    Purpose Of Review: The ANCA-associated vasculitides are a group of small vessel vasculitides characterized by autoantibodies recognizing the neutrophil cytoplasmic antigens PR3 and MPO. We examine the current clinical and molecular immunology understanding of ANCA-associated vasculitides and discuss the current needs in our understanding of the pathogenic mechanisms of these rare diseases.

    Recent Findings: The majority of efforts to understand the pathogenesis of these diseases have focused on dissecting neutrophil biology because the neutrophil is the primary expressor of ANCA autoantigens. Read More

    Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse: A case report.
    Medicine (Baltimore) 2017 Mar;96(13):e6024
    aDivisione di Clinica Medica bDivisione di Anestesia e Rianimazione cDivisione di Radiologia dDivisione di Anatomia Patologica, Ospedale San Gerardo, Monza eUniversity of Milano-Bicocca and Istituto di Ricerca a Carattere Scientifico IRCCS Multimedica, Sesto San Giovanni, Milano, Italy.
    Rationale: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitis affecting small- and medium-sized blood vessels, mostly involving lung and kidney.

    Patient Concerns: We report the case of a 33-year-old man that presented with acute respiratory distress syndrome caused by alveolar hemorrhage.

    Diagnoses: Aggressive GPA presenting with diffuse alveolar hemorrhage and multiorgan involvement. Read More

    A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature.
    Case Rep Rheumatol 2017 28;2017:2421760. Epub 2017 Feb 28.
    UCLA-Olive View Rheumatology Program, Division of Rheumatology, Olive View-UCLA Medical Center, 14445 Olive View Drive, 2B182, Sylmar, CA 91342, USA.
    This paper describes one patient with Antineutrophil Cytoplasmic Antibody- (ANCA-) associated vasculitis who initially presented with multiple ischemic fingers and toes. On further evaluation, the patient was also found to have pulmonary-renal involvement and episcleritis. The diagnosis was supported with a positive cANCA (anti-proteinase 3) and a bronchoscopy consistent with diffuse alveolar hemorrhage. Read More

    Systemic Lupus Erythematosus and hearing disorders: Literature review and meta-analysis of clinical and temporal bone findings.
    J Int Med Res 2017 Jan 1:300060516688600. Epub 2017 Jan 1.
    2 Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Italy.
    Objective This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Read More

    Chronic nasal Staphylococcus aureus carriage identifies a subset of newly diagnosed granulomatosis with polyangiitis patients with high relapse rate.
    Rheumatology (Oxford) 2017 Feb 20. Epub 2017 Feb 20.
    Department of Internal Medicine, Vaasa Central Hospital, Vaasa, Finland.
    Objective.: The aim of this study was to evaluate whether chronic nasal carriage of Staphylococcus aureus (SA) is related to relapses in patients with newly diagnosed ANCA-associated vasculitis (AAV).

    Methods. Read More

    Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients.
    Rheumatology (Oxford) 2017 Feb 16. Epub 2017 Feb 16.
    Service de Radiologie B.
    Objectives.: Specific cardiac involvement in granulomatosis with polyangiitis (GPA) is probably underestimated since many of these conditions are subclinical. The objective of this study was to assess the prevalence and patterns of cardiac abnormalities detected by cardiac MRI (CMRI) in patients with GPA. Read More

    Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA negative vasculitis.
    Clin Exp Rheumatol 2017 Mar 23. 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 having 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 15. 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

    Clinical features and long-term outcomes of 105 granulomatosis with polyangiitis patients: A single center experience from north India.
    Int J Rheum Dis 2017 Mar 24. Epub 2017 Mar 24.
    Department of Immunopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
    Aim: To describe the clinical features, treatment and long-term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA).

    Methods: Clinical details, Birmingham Vasculitis Activity Score (BVAS-v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories. 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

    Contrast-enhanced ultrasound and computed tomography findings of granulomatosis with polyangiitis presenting with multiple intrarenal microaneurysms: A case report.
    Clin Imaging 2017 Mar 9;43:144-147. Epub 2017 Mar 9.
    Department of Radiology, Wonkwang University, College of Medicine and Hospital, 895 Muwang-Ro, Iksan, Jeonlabuk-do 54538, Republic of Korea.
    Granulomatosis with polyangiitis (GPA) is a systemic disorder that affects small- and medium- sized vessels in many organs. Although the kidneys are the second most commonly involved organ in patients with GPA, its manifestation as multiple intrarenal aneurysms is rare. We report an unusual manifestation of GPA with multiple intrarenal microaneurysms, as demonstrated by contrast-enhanced ultrasound and computed tomography. Read More

    [Subglottic stenosis as the initial manifestation of Wegener's granulomatosis in a teenager. Case report].
    Arch Argent Pediatr 2017 Apr;115(2):e120-e125
    Hospital General de Niños "Dr. Pedro de Elizalde", Ciudad Autónoma de Buenos Aires, Argentina.
    Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Read More

    Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis.
    Curr Opin Rheumatol 2017 May;29(3):248-253
    University of Michigan, Ann Arbor, Michigan, USA.
    Purpose Of Review: The antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitides are a group of rare systemic diseases. The past several years have seen major therapeutic advances in the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The success rate in induction of remission is high, but reducing the high incidence of relapses remains a therapeutic challenge. Read More

    Ulcerative granuloma of the eyelid as the initial manifestation of granulomatosis with polyangiitis (Wegener's granulomatosis): A case report.
    Orbit 2017 Mar 17:1-4. Epub 2017 Mar 17.
    a Department of Ophthalmology, Oculoplastics & Orbit , Bellvitge University Hospital , L'Hospitalet de Llobregat, Barcelona , Spain.
    A 56-year-old-man presented a 2-month history of chalazion in the eyelids without response to treatment and with an inconclusive biopsy. Laboratory results confirmed the presence of Enterobacter cloacae and Streptococcus gordonii infection. Despite appropriate intravenous antibiotic treatment, clinical worsening was observed. Read More

    Accessory left gastric artery aneurysms in granulomatosis with polyangiitis: a case report and literature review.
    Nagoya J Med Sci 2017 Feb;79(1):75-83
    Department of Surgery, Tosei General Hospital, Seto, Japan.
    Aneurysm formation is a potential complication of granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis. It is a very rare complication, but immediate diagnosis and therapy should be performed because an aneurysm can be life-threatening if it ruptures. An accessory left gastric artery (ALGA) is also a rare variant gastric artery that may obtain its blood supply from the left hepatic artery and left gastric artery. Read More

    [Refractory form of orbital granulomatosis with polyangiitis: A clinical and morphological study].
    Arkh Patol 2017 ;79(1):56-60
    Research Institute of Eye Diseases, Moscow, Russia.
    Orbital granulomatosis with polyangiitis (Wegener's granulomatosis, GPA), which is characterized by granulomatous inflammation with small-vessel vasculitis, can develop in local and generalized forms of the disease. The introduction of current immunosuppressive therapy regimens has improved the prognosis of the disease; however, there are immunosuppressive treatment-refractory forms of GPA, the morphology of which has been inadequately investigated. The paper describes a clinical case of refractory GPA involving the orbit, as evidenced by histological and immunohistochemical examinations. Read More

    Refractory optic perineuritis due to granulomatosis with polyangiitis successfully treated with methotrexate and mycophenolate mofetil combination therapy.
    Eur J Rheumatol 2017 Mar 1;4(1):70-72. Epub 2017 Mar 1.
    Department of Internal Medicine, Teikyo University School of Medicine, Tokyo, Japan.
    Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Read More

    Immune stimulatory effects of neutrophil extracellular traps in granulomatosis with polyangiitis.
    Clin Exp Rheumatol 2017 Mar 8. Epub 2017 Mar 8.
    Klinikum Bad Bramstedt, Rheumatology and Immunology, Bad Bramstedt; and Rheumazentrum Schleswig-Holstein Mitte, Neumünster, Germany.
    Objectives: The aim of this study was to analyse the role of netting neutrophils in the pathogenesis of granulomatosis with polyangiitis (GPA), especially their interplay with peripheral blood mononuclear cells (PBMCs).

    Methods: The amount of cell-free DNA (cfDNA) was determined in sera from GPA patients (pairs active/inactive state of disease, n=18) and from healthy controls (HCs, n=10). Furthermore, we performed in vitro incubation experiments using PBMCs and NETs from patients and HCs for accessing the effect of NETs on PBMC behaviour. Read More

    IgA antibodies to myeloperoxidase in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
    Clin Exp Rheumatol 2017 Mar 1. 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 multi-center 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

    ANCA Associated Mononeuritis Multiplex with Overlap in Vasculitic Syndromes.
    J Clin Diagn Res 2017 Jan 1;11(1):OD01-OD03. Epub 2017 Jan 1.
    Assistant Professor, Department of Neuropathology, NIMHANS , Bengaluru, Karnataka, India .
    Mononeuritis multiplex is a common manifestation of many illnesses which includes Hansen's disease and certain types of systemic vasculitis. The Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA). Read More

    A Cardiac Graft from a Donor with Granulomatosis with Polyangiitis-A Case Report.
    Thorac Cardiovasc Surg Rep 2017 Jan 19;6(1):e3-e4. Epub 2016 Dec 19.
    Department of Cardiovascular Surgery, University of Schleswig-Holstein, Campus Kiel, Kiel, Germany.
    Organ shortage unavoidably leads to shifting strategies in modern transplantation medicine. Experiences with specific comorbidities in terms of organ transplantation therefore have to be made. We report a case of a 51-year-old male patient with successful orthotopic heart transplantation from a donor with granulomatosis with polyangiitis. Read More

    Five factor score more than 1 is associated with relapse during the first 2 year-follow up in patients with eosinophilic granulomatosis with polyangiitis.
    Int J Rheum Dis 2017 Mar 5. Epub 2017 Mar 5.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea.
    Aim: We investigated what variables at diagnosis might be associated with the relapse of eosinophilic granulomatosis with polyangiitis (EGPA) and whether five factor score (FFS) at diagnosis might predict it.

    Methods: We reviewed the medical records of 30 patients with EGPA having results of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA) and proteinase 3 (PR3)-ANCA and having achieved remission during the first therapeutic regimen administration within follow-up duration for at least more than 2 years. Clinical manifestations, the presence of relapse and items for FFS at diagnosis were collected and compared among groups. Read More

    Granulomatosis with polyangiitis involving the epiglottis.
    Respirol Case Rep 2017 May 1;5(3):e00226. Epub 2017 Mar 1.
    Second Division, Department of Internal Medicine Hamamatsu University School of Medicine Hamamatsu Japan.
    Granulomatosis with polyangiitis (GPA) frequently involves the upper respiratory tracts, but involvement of the epiglottis is extremely rare. This report describes a patient initially presenting with dysphagia and increasing stridor due to epiglottitis. Bronchoscopy showed swelling of the epiglottis with partly whitish nodular lesions, with biopsy specimens showing neutrophil infiltration and necrosis. Read More

    Granulomatosis with Polyangiitis (Wegener Granulomatosis) with Unusual Presentation.
    Mymensingh Med J 2017 Jan;26(1):205-207
    Dr MA Mannan, Consultant, ICU, Dr Sirajul Islam Medical College & Hospital, Dhaka, Bangladesh.
    We came across a 32 years old male admitted in our hospital with prolonged low-grade fever, haemoptysis, leg swelling, weight loss, purpuric rashes and malaena. He received anti-TB treatment at another hospital without any improvement. He was pale with bullous and purpuric lesions over legs and feet. Read More

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