Search our Database of Scientific Publications and Authors

I’m looking for a

    7591 results match your criteria Wegener Granulomatosis

    1 OF 152

    [Neurological disorders in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].
    Zh Nevrol Psikhiatr Im S S Korsakova 2016 ;116(10):93-102
    Pirogov City Clinical Hospital, Moscow, Russia.
    Eosinophilic granulomatosis with polyangiitis - EGPA (Churg-Strauss syndrome) is a rare autoimmune disorder. The pathogenesis of the disease includes production of anti-neutrophil cytoplasmic antibodies directed against myeloperoxidase with the development of small-vessel necrotizing vasculitis and eosinophilic infiltration of organs. The involvement of peripheral and central nervous system is observed in more than 3/4 of cases. Read More

    [Clinical Course of an Early Childhood-Onset Granulomatosis with Polyangiitis].
    Dtsch Med Wochenschr 2017 Jun 21;142(12):904-908. Epub 2017 Jun 21.
    Sektion Nephrologie, Klinik für Endokrinologie und Nephrologie, Department für Innere Medizin, Neurologie und Dermatologie, Universitätsklinikum Leipzig AöR.
    History A 26-year-old male patient presented with an eight-week history of unspecific symptoms such as weight loss and fever. Besides, he also suffered from haemoptysis, cough, and arthralgia. Since the age of twelve years, the patient has been treated for Wegner's granulomatosis. Read More

    [The frequency of ophthalmologic manifestations of granulomatosis with polyangiitis (Wegener's) and their relationship to systemic diseases].
    Ter Arkh 2017 ;89(5):69-73
    M.V. Lomonosov Moscow State University, Moscow, Russia.
    Aim: To estimate the frequency of lesions in the organ of vision in granulomatosis with polyangiitis (GPA) (Wegener's) and to determine their relationship to systemic diseases.

    Subjects And Methods: The retrospective study enrolled 218 patients followed up at the E.M. Read More

    Rituximab Induction and Maintenance Treatment in Patients with Scleritis and Granulomatosis with Polyangiitis (Wegener's).
    Ocul Immunol Inflamm 2017 Jun 19:1-8. Epub 2017 Jun 19.
    a Massachusetts Eye Research and Surgery Institution (MERSI) , Waltham , Massachusetts , USA.
    Aims: To evaluate the efficacy and safety of rituximab (RTX) induction and maintenance treatment for patients with scleritis and granulomatosis with polyangiitis (GPA), Wegener's.

    Methods: Nine patients (12 eyes) with scleritis with GPA who did not respond to corticosteroids and more than one immunosuppressive agent who received ongoing maintenance RTX treatment were identified. Demographics and outcome measures were recorded. Read More

    Vasculitis for the internist: focus on ANCA-associated vasculitis.
    Intern Emerg Med 2017 Jun 16. Epub 2017 Jun 16.
    Department of Internal Medicine, Hôpital Cochin, APHP, Université Paris Descartes, 27, rue du faubourg Saint-Jacques, 75679, Paris Cedex 14, France.
    Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality. Read More

    Chemokine receptor co-expression reveals aberrantly distributed TH effector memory cells in GPA patients.
    Arthritis Res Ther 2017 Jun 14;19(1):136. Epub 2017 Jun 14.
    Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands.
    Background: Persistent expansion of circulating CD4(+) effector memory T cells (TEM) in patients with granulomatosis with polyangiitis (GPA) suggests their fundamental role in disease pathogenesis. Recent studies have shown that distinct functional CD4(+) TEM cell subsets can be identified based on expression patterns of chemokine receptors. The current study aimed to determine different CD4(+) TEM cell subsets based on chemokine receptor expression in peripheral blood of GPA patients. Read More

    The "4 plus 2" rituximab protocol makes maintenance treatment unneeded in patients with refractory ANCA-associated vasculitis: A 10 year observation study.
    Oncotarget 2017 May 23. Epub 2017 May 23.
    Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
    Background: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Read More

    [Granulomatous reaction pattern of the skin : Interstitial granulomatous dermatitis - lymphoma - vasculitis].
    Hautarzt 2017 Jun 12. Epub 2017 Jun 12.
    Konsultations- und Referenzzentrum für Vaskulitis-Diagnostik, MVZ am Marienkrankenhaus gGmbH, Hamburg, Deutschland.
    A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Read More

    Molecularly targeted agents in oculoplastic surgery.
    Curr Opin Ophthalmol 2017 Jun 8. Epub 2017 Jun 8.
    Section of Ophthalmology, Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
    Purpose Of Review: Significant advances have been made in oncology and rheumatology with the introduction of molecularly targeted agents (MTAs). MTAs consist of monoclonal antibodies and small molecule inhibitors. The purpose of this manuscript is to review the recent applications of MTAs to orbital, lacrimal, and eyelid disease. Read More

    Uncommon presentation of granulomatosis with polyangiitis in association with subclinical coeliac disease.
    Clin Exp Rheumatol 2017 Jun 9. Epub 2017 Jun 9.
    Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Greece.
    ****************************************************************************. Read More

    Pulmonary-Limited Granulomatosis with Polyangiitis Coexisting with Mixed Connective Tissue Disease.
    Tohoku J Exp Med 2017 ;242(2):109-114
    Department of Respiratory Medicine and Infectious Diseases, Oita University Faculty of Medicine.
    Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis. 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

    Imaging diagnosis of orbital Wegener granulomatosis: A rare case report.
    Medicine (Baltimore) 2017 Jun;96(23):e6904
    aDepartment of Radiology, The First Affiliated Hospital of Kunming Medical University, Kunming bDepartment of Radiology, The First Affiliated Hospital of Dali University, Dali cDepartment of Ophthalmology, The First Affiliated Hospital of Dali University, Dali, China.
    Introduction: Wegener granulomatosis (WG) is a rare idiopathic autoimmune disease causing necrotizing granulomatous vasculitis. Whether as the first symptom or as part of systemic changes, ocular manifestations in WG patients are not specific. Any part of the eyes can be affected, with the anterior segment and orbit most commonly involved. Read More

    Eosinophilic angiitis presenting with a true, fusiform, temporal artery aneurysm.
    Clin Case Rep 2017 Jun 8;5(6):769-773. Epub 2017 Apr 8.
    Department of RheumatologyQueen Elizabeth Hospital BirminghamMendelsohn Way, EdgbastonBirminghamB15 2THUK.
    Temporal artery aneurysm is a rare cause of temporal artery swelling in the absence of preceding trauma. Vasculitis other than giant cell arteritis, such as eosinophilic granulomatosis with polyangiitis, should be considered in such cases and a careful assessment of other medium-sized arteries undertaken. Read More

    Successful treatment of granulomatosis with polyangiitis with hydropneumothorax using corticosteroids and immunosuppressant.
    Exp Ther Med 2017 Jun 8;13(6):3586-3590. Epub 2017 May 8.
    Department of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, P.R. China.
    Pneumothorax and pleural effusion is a rare and serious complication of granulomatosis with polyangiitis (GPA). The present study reported a case with a history of sinusitis for 20 years, dry cough for three years and exacerbated purulent nasal discharge and recurrent skin ulcers for two years. The patient experienced sudden difficulty in breathing two months prior to presentation. Read More

    A case report of the orbit, ocular association and the lung in granulomatosis with polyangiitis: A diagnostic challenge.
    Exp Ther Med 2017 Jun 28;13(6):3337-3340. Epub 2017 Apr 28.
    Department of Ophthalmology, The First Hospital of Jilin University, Changchun, Jilin 130021, P.R. China.
    Granulomatosis with polyangiitis (GPA) is a systemic form of vasculitis that involves small to medium sized vessels and is associated with high morbidity and mortality. GPA presents a continuous and difficult clinical diagnostic concern, due to the rarity of the disease and the diversity of the manifestations. This case report discusses the unusual symptoms presented by a particular patient, discusses these manifestations and explains how the final diagnosis was identified as GPA. Read More

    Cutaneous Manifestations of ANCA-Associated Small Vessels Vasculitis.
    Clin Rev Allergy Immunol 2017 Jun 3. Epub 2017 Jun 3.
    Division of Rheumatology, ASST Pini, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy.
    Skin lesions are frequent manifestations of underlying systemic conditions, including systemic autoimmune vasculitis. In particular, anti-neutrophil cytoplasmic antibodies (ANCA) are associated with distinct forms of vasculitis characterized by inflammatory cell infiltration of the walls of small and medium-sized vessels leading to vascular destruction and tissue necrosis. ANCA-associated vasculitis is rare and systemic diseases, which can be classified based on different distribution of vascular inflammation and presence or absence of granulomatosis and asthma. Read More

    Cardiac involvements in hypereosinophilia-associated syndrome: Case reports and a little review of the literature.
    Echocardiography 2017 Jun 1. 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

    Echocardiographic Assessment in Patients with Granulomatosis with Polyangiitis.
    Adv Exp Med Biol 2017 Jun 2. Epub 2017 Jun 2.
    Department of Family Medicine, Internal and Metabolic Diseases, Warsaw Medical University, 1A Banacha Street, 02-097, Warsaw, Poland.
    Granulomatosis with polyangiitis (GPA) is one of the most common forms of systemic vasculitis, which usually involves the upper and lower respiratory tract, but it may affect also multiple organs. The aim of the study was an echocardiographic evaluation of cardiac involvement in GPA patients during remission. Eighty eight patients with GPA were evaluated in the study. Read More

    Longterm Prognosis of 121 Patients with Eosinophilic Granulomatosis with Polyangiitis in Japan.
    J Rheumatol 2017 Jun 1. 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. N. Tsurikisawa, MD, Department of Respirology, National Hospital Organization Saitama National Hospital, and Department of Allergy and Respirology, National Hospital Organization Sagamihara National Hospital; C. Oshikata, MD, Department of Respirology, National Hospital Organization Saitama National Hospital, and Department of Allergy and Respirology, National Hospital Organization Sagamihara National Hospital; A. Kinoshita, MD, Department of Allergy and Respirology, National Hospital Organization Sagamihara National Hospital; T. Tsuburai, MD, Department of Allergy and Respirology, National Hospital Organization Sagamihara National Hospital; H. Saito, PhD, Department of Allergy and Respirology, National Hospital Organization Sagamihara National Hospital. Address correspondence to Dr. N. Tsurikisawa, Department of Respirology, National Hospital Organization Saitama National Hospital, 2-1 Suwa, Wako, Saitama, Japan 351-0102. E-mail: Accepted for publication April 12, 2017.
    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

    Myocarditis in auto-immune or auto-inflammatory diseases.
    Autoimmun Rev 2017 May 29. Epub 2017 May 29.
    Département de Médecine Interne et Immunologie Clinique, Assistance Publique-Hôpitaux de Paris (AP-HP), Groupe Hospitalier Pitié-Salpêtrière, F-75013 Paris, France; Département Hospitalo-Universitaire I2B, UPMC Univ Paris 06, F-75013 Paris, France; INSERM, UMR 7211, F-75005 Paris, France; CNRS, UMR 7211, F-75005 Paris, France; INSERM, UMR_S 959, F-75013 Paris, France. Electronic address:
    Myocarditis is a major cause of heart disease in young patients and a common precursor of heart failure due to dilated cardiomyopathy. Some auto-immune and/or auto-inflammatory diseases may be accompanied by myocarditis, such as sarcoidosis, Behçet's disease, eosinophilic granulomatosis with polyangiitis, myositis, and systemic lupus erythematosus. However, data concerning myocarditis in such auto-immune and/or auto-inflammatory diseases are sparse. Read More

    ANCA-associated vasculitis with renal involvement.
    J Nephrol 2017 May 30. Epub 2017 May 30.
    Divisione di Nefrologia e Dialisi-Padiglione Croff, Fondazione Ca' Granda IRCCS Ospedale Maggiore Policlinico di Milano, Via della Commenda 15, 20122, Milan, Italy.
    Systemic vasculitis is a rare but severe group of diseases characterized by inflammation and necrosis of blood vessels. The size of the vessel affected varies among the different forms of vasculitis and there are three main subgroups: large, medium and small vessel vasculitis. Among small vessel vasculitis, the antineutrophil cytoplasmic antibody (ANCA)-associated forms are of particular importance. Read More

    Severe microscopic polyangiitis with unilateral vocal cord paralysis as initial manifestation.
    Colomb Med (Cali) 2017 Mar 30;48(1):32-34. Epub 2017 Mar 30.
    Instituto Nacional de Enfermedades Respiratorias, Ciudad Mexico , Mexico.
    Case Description: A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement.

    Clinical Findings: Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis. Renal biopsy confirmed an ANCA-associated vasculitis. 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

    Comparison of enzyme-linked immunosorbent assay and rapid chemiluminescent analyser in the detection of myeloperoxidase and proteinase 3 autoantibodies.
    Pathology 2017 Jun 23;49(4):413-418. Epub 2017 May 23.
    Department of Immunology, The Canberra Hospital, Australia; ACT Pathology, The Canberra Hospital, Australia; ANU Medical School, Australian National University, Canberra, Australia; Centre for Personalised Immunology, John Curtin School of Medical Research, ANU, Canberra, ACT, Australia. Electronic address:
    Antibodies to myeloperoxidase (MPO) and proteinase 3 (PR3) are vital in the diagnosis and management of ANCA-associated vasculitis. A chemiluminescent immunoassay (CLIA; Quanta Flash) provides MPO and PR3 antibody results in 30 minutes, which is much faster than enzyme-linked immunosorbent assay (ELISA). We compared the performance of ELISA (Orgentec) and CLIA (Quanta Flash) for MPO and PR3 antibody quantitation on 303 samples, comprising 196 consecutive samples received in a single diagnostic laboratory over a 3 month period, and 107 samples collected from 42 known vasculitis patients over a 40 month period. Read More

    Proteinase 3: the odd one out that became an autoantigen.
    J Leukoc Biol 2017 May 25. Epub 2017 May 25.
    Institut National de la Santé et de la Recherche Médicale, U1016, Institut Cochin, Paris, France;
    Neutrophils are critical in the defense against bacterial and fungal pathogens, and they also modulate the inflammatory process. The areas where neutrophils are studied have expanded from the restricted field of antibacterial defense to the modulation of inflammation and finally, to fine-tuning immune responses. As a result, recent studies have shown that neutrophils are implicated in several systemic autoimmune diseases, although exactly how neutrophils contribute to these diseases and the molecular mechanisms responsible are still under investigation. Read More

    Granulomatosis with Polyangiitis Presenting with Acute Aortic and Mitral Regurgitation: Case Report and Big-Data Analysis.
    J Heart Valve Dis 2017 Jan;26(1):103-106
    Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center, Cleveland, Ohio, USA. Electronic correspondence:
    Granulomatosis with polyangiitis (GPA) is a rare vasculitis that can have multisystem involvement, though cardiac involvement is very rare. The case is described of a 53-year-old woman who presented with acute aortic and mitral valve regurgitation requiring surgical intervention. Pathology from the excised aortic valve showed geographic necrosis concerning for GPA. Read More

    A multicentre study to improve clinical interpretation of proteinase-3 and myeloperoxidase anti-neutrophil cytoplasmic antibodies.
    Rheumatology (Oxford) 2017 May 24. Epub 2017 May 24.
    Central Diagnostic Laboratory, Maastricht University Medical Center, Maastricht, The Netherlands.
    Objective.: The objective of this multicentre study was to improve the clinical interpretation of PR3- and MPO-ANCAs as an adjunct for the diagnosis of ANCA-associated vasculitis (AAV) by defining thresholds and test result intervals based on predefined specificities and by calculating test result interval-specific likelihood ratios (LRs).

    Methods. 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

    Pituitary dysfunction in granulomatosis with polyangiitis.
    Pituitary 2017 May 24. Epub 2017 May 24.
    Department of Endocrinology, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
    Purpose: Granulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Read More

    Pathophysiology of ANCA-associated Vasculitis.
    Adv Anat Pathol 2017 Jul;24(4):226-234
    Departments of *Pathology and Laboratory Medicine †Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Kingdom of Saudi Arabia.
    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is characterized as inflammation of small-sized to medium-sized blood vessels and encompasses several clinicopathologic entities including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited ANCA-associated vasculitis. Over the past several decades, significant progress has been made in understanding the pathophysiology of ANCA-associated vasculitis. Although neutrophils contain a multitude of granular proteins, clinically significant autoantibodies are only recognized against myeloperoxidase and proteinase 3, both of which are present in the azurophilic granules. Read More

    Granulomatosis with polyangiitis in Tunisia.
    Reumatismo 2017 May 22;69(1):23-29. Epub 2017 May 22.
    Department of Internal Medicine, Medical Faculty of Tunis.
    Granulomatosis with polyangiitis (GPA) is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Read More

    Oculo-otological Manifestations in a Case of Granulomatosis with Polyangiitis.
    J Assoc Physicians India 2017 Apr;65(4):84
    Ex Resident, Department of Medicine, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra.
    A 44 year old lady presented with acute onset of loss of vision in the right eye and cough with mucopurulent expectoration for two months. Ophthalmic examination revealed central retinal artery occlusion (CRAO). Chest radiograph showed multiple cavitatory nodules with fluid levels. Read More

    [Combination therapy of refractory localized granulomatosis with polyangiitis and orbital involvement].
    Vestn Oftalmol 2017 ;133(2):86-91
    Research Institute of Eye Diseases, 11 A,B Rossolimo St., Moscow, Russian Federation, 119021; I.M. Sechenov First Moscow State Medical University, 2, str. 4 Bol'shaya Pirogovskaya St., Moscow, Russian Federation, 119991.
    Granulomatous orbital cellulitis is one of the most common ocular manifestations of granulomatosis with polyangiitis (Wegener's). The process is often refractory to conventional immunosuppressive therapy and requires a more radical treatment approach. However, surgical experience with this type of patients is limited. Read More

    B cell repopulation kinetics after rituximab treatment in ANCA-associated vasculitides compared to rheumatoid arthritis, and connective tissue diseases: a longitudinal observational study on 120 patients.
    Arthritis Res Ther 2017 May 18;19(1):101. Epub 2017 May 18.
    Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, Hugstetterstrasse 55, 79106, Freiburg, Germany.
    Background: B cell depletion with rituximab (RTX) is approved for treatment of rheumatoid arthritis (RA) and ANCA-associated vasculitides (AAV). Recently, RTX has been shown to be effective in AAV maintenance therapy, but an optimal RTX treatment schedule is unknown and the time to B cell repopulation after RTX has not been studied.

    Methods: Retrospective single-center analysis of B cell repopulation in patients with AAV, RA or connective tissue disease (CTD) treated with RTX. Read More

    M2 macrophage is the predominant phenotype in airways inflammatory lesions in patients with granulomatosis with polyangiitis.
    Arthritis Res Ther 2017 May 18;19(1):100. Epub 2017 May 18.
    Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
    Background: Macrophages may present two distinct phenotypes indicated as M1 and M2 under different stimuli. M1 and M2 macrophages have divergent functions that range from enhancement of inflammation for M1 to tissue repair and remodeling for M2 macrophages. The objective of this study was to evaluate the distribution of M1 and M2 macrophage phenotypes in biopsies from the airways of patients with active granulomatosis with polyangiitis (GPA) and to analyze their associations with T and B cells in those biopsies, and with nasal carriage of Staphylococcus aureus, disease parameters and therapy. Read More

    Vasculitis following influenza vaccination: A review of the literature.
    Curr Rheumatol Rev 2017 May 17. Epub 2017 May 17.
    Department of Pediatrics, Niigata City General Hospital, Niigata. Japan.
    Background: Influenza vaccine is safe and effective for the general population as well as for patients with autoimmune diseases. However, although rare, vasculitis has been reported as an adverse event following influenza vaccination.

    Object: The aims of this literature review were to identify patients who developed vasculitis following influenza vaccination and to clarify the clinical manifestations of vasculitis in these patients. Read More

    Targeting Eosinophils in Eosinophilic Granulomatosis with Polyangiitis.
    N Engl J Med 2017 05;376(20):1985-1986
    From Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, and National Institute for Health Research Southampton Biomedical Research Centre, Southampton, United Kingdom (R.D.); and Firestone Institute for Respiratory Health, St. Joseph's Healthcare, and the Department of Medicine, Michael G. DeGroote School of Medicine, McMaster University, Hamilton, ON, Canada (P.M.O.).

    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

    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

    Clinically isolated aortitis: pitfalls, progress, and possibilities.
    Cardiovasc Pathol 2017 Apr 23;29:23-32. Epub 2017 Apr 23.
    Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:
    Non-infectious aortitis may be caused by several distinct systemic rheumatologic diseases. In some patients, aortitis is identified either pathologically or radiologically in the absence of clinical evidence of a systemic vasculitis. By consensus nomenclature, such cases are referred to as clinically isolated aortitis (CIA). Read More

    CT-P10 (Truxima™): A Rituximab Biosimilar.
    BioDrugs 2017 Jun;31(3):275-278
    Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.
    CT-P10 (Truxima™) is the first biosimilar of the reference monoclonal anti-CD20 antibody rituximab. It is approved for use in all indications for which reference rituximab is approved, including follicular lymphoma (FL), diffuse large B-cell non-Hodgkin's lymphoma, chronic lymphocytic leukaemia, rheumatoid arthritis (RA), granulomatosis with polyangiitis and microscopic polyangiitis. CT-P10 has similar physicochemical and pharmacodynamic properties to those of reference rituximab, and the pharmacokinetic biosimilarity of the agents has been shown in patients with RA or FL. Read More

    1 OF 152