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    Seronegative granulomatosis with polyangiitis presenting with multiple cranial nerve palsies.
    Neuropathology 2017 Nov 14. Epub 2017 Nov 14.
    Department of Pathology, Seoul National University Hospital, College of Medicine, Seoul, Republic of Korea.
    Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. Read More

    Eosinophilic granulomatosis with polyangiitis complicated by subarachnoid hemorrhage and coronary vasculitis: a case report and review of the literature.
    Rheumatol Int 2017 Nov 10. Epub 2017 Nov 10.
    Department of Internal Medicine (IV), Osaka Medical College, 2-7 Daigaku-machi, Takatsuki, Osaka, 569-8686, Japan.
    Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Read More

    The 2017 Doyne Lecture: the orbit as a window to systemic disease.
    Eye (Lond) 2017 Nov 10. Epub 2017 Nov 10.
    Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, and Centre for Eye Research Australia, University of Melbourne, Melbourne, Victoria, Australia.
    A very large number of disorders affect the orbit, and many of these occur in the setting of systemic disease. This lecture covers selected aspects of orbital diseases with systemic associations in which the author has a particular clinical or research interest. Spontaneous orbital haemorrhage often occurs in the presence of bleeding diatheses. Read More

    Patient perceptions of glucocorticoids in anti-neutrophil cytoplasmic antibody-associated vasculitis.
    Rheumatol Int 2017 Nov 9. Epub 2017 Nov 9.
    Division of Rheumatology, Departments of Medicine and Epidemiology, University of Pennsylvania, Philadelphia, PA, USA.
    Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are multisystem diseases of small blood vessels, collectively known as the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This study explores the patient's perspective on the use of glucocorticoids, which are still a mainstay of treatment in AAV. Patients with AAV from the UK, USA, and Canada were interviewed, using purposive sampling to include a range of disease manifestations and demographics. Read More

    Rheumatoid factor false positivity in patients with ANCA-associated vasculitis not having medical conditions producing rheumatoid factor.
    Clin Rheumatol 2017 Nov 8. Epub 2017 Nov 8.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
    We investigated the rate of rheumatoid factor (RF) false positivity at diagnosis and its influence on clinical and prognostic features and rheumatoid arthritis (RA) development during the follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients without RA or other medical conditions triggering RF false positivity. We reviewed the medical records of 128 AAV patients. We divided patients with AAV and each variant into two groups according to RF positivity and compared variables between the two groups. Read More

    Cryotherapy to treat and prevent airway stenosis in a patient with granulomatosis with polyangiitis.
    Pediatr Pulmonol 2017 Nov 3. Epub 2017 Nov 3.
    Icahn School of Medicine at Mount Sinai, Departments of Pediatrics (Kravis Children's Hospital), New York, New York.
    We report our use of cryotherapy delivered via flexible bronchoscopy in a 15-year old girl with granulomatosis with polyangiitis to both treat established airway stenosis and prevent multi-level progression of disease. Read More

    Subglottic Stenosis in Granulomatosis With Polyangiitis: The Role of Laryngotracheal Resection.
    Ann Thorac Surg 2017 Nov 1. Epub 2017 Nov 1.
    Division of Thoracic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:
    Background: Granulomatosis with polyangiitis (GPA) is associated with development of subglottic stenosis in about one-fourth of all patients. Although endoscopic management is the primary treatment method for tracheobronchial stenosis, some patients have refractory disease, and tracheostomy is required. It is unclear if laryngotracheal resection and reconstruction (LTRR) can be safely performed in patients with GPA. Read More

    A Case Report on Suspected Levamisole-Induced Pseudovasculitis.
    WMJ 2017 02;116(1):37-9
    Introduction: Levamisole-induced pseudovasculitis should be considered in patients with inconsistent anti-neutrophil cytoplasmic antibodies (ANCA) pattern and history of cocaine use.

    Case Presentation: A 50-year-old man presented to the emergency department with symptoms of bilateral pulmonary emboli. His hospital course was complicated by multiple end organ failure, which improved dramatically with prednisone. Read More

    Eosinophilic Myocarditis: Characteristics, Treatment, and Outcomes.
    J Am Coll Cardiol 2017 Nov;70(19):2363-2375
    San Raffaele Vita-Salute University, Milan, Italy; Transplant Center and "De Gasperis" Cardio Center, Niguarda Hospital, Milan, Italy. Electronic address:
    Background: Eosinophilic myocarditis (EM) is an acute life-threatening inflammatory disease of the heart. Neither large case series nor clinical trials on this specific myocarditis have been reported.

    Objectives: Based on a systematic revision of all published histologically proven cases, this study aimed to describe the clinical presentation, treatment, and outcome of EM. Read More

    Diffuse Alveolar Hemorrhage Developing Immediately after Immunosuppressive Treatments in a Patient with Granulomatosis with Polyangiitis who had Pulmonary Nodules.
    Intern Med 2017 Nov 1. Epub 2017 Nov 1.
    Unit of Advanced Preventive Medical Sciences, Departments of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan.
    A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the detection of high MPO-ANCA, vasculitis and granulomas in a lung biopsy specimen and crescentic glomerulonephritis in a kidney biopsy specimen. Soon after the initiation of intravenous methylprednisolone pulse therapy (mPSL pulse) and intravenous cyclophosphamide pulse therapy (IVCY), the patient experienced cough and hemoptysis. Based on emerging anemia and bilateral diffuse lung consolidation on computed tomography, we judged that diffuse alveolar hemorrhage (DAH) was complicated by GPA. Read More

    Granulomatosis with polyangiitis (Wegener's granulomatosis): a rare variant of sudden natural death.
    Int J Legal Med 2017 Oct 31. Epub 2017 Oct 31.
    Institute of Legal Medicine, University Hospital Münster, Röntgenstraße 23, 48149, Münster, Germany.
    Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is the most common form of life-threatening small-vessel vasculitis. Although its exact pathogenesis remains unclear, GPA is believed to belong to the wide complex of autoimmune diseases due to the presence of anti-neutrophil cytoplasmatic antibodies with cytoplasmic staining pattern (c-ANCA) that is expressed in activated neutrophils. GPA predominantly manifests at the upper and lower respiratory tract and the kidneys, but the impairment of multiple organ systems is possible as well. Read More

    [Granulomatosis with polyangiitis associated with the cytoplasmic antineutrophil antibodies].
    Vestn Otorinolaringol 2017 ;82(5):52-55
    L.I. Sverzhevskiy Research Institute of Clinical Otorhinolaryngology, Moscow Health Department, Moscow, Russia, 117152.
    The objective of the present work was to analyze the clinical case of granulomatosis with polyangiitis associated with the presence of cytoplasmic antineutrophil antibodies. We considered the medical records of the patient presenting with this condition. It was shown that systemic vasculitis had a polymorphic clinical picture; its diagnostics and treatment encountered serious difficulties. Read More

    A cohort study of comorbidity in patients with granulomatosis with polyangiitis.
    Rheumatology (Oxford) 2017 Oct 23. Epub 2017 Oct 23.
    Boston Collaborative Drug Surveillance Program, Boston University School of Public Health, Lexington.
    Objectives: To evaluate the associations between granulomatosis with polyangiitis (GPA) and a wide range of comorbidities in patients with GPA compared with population-based non-vasculitis patients.

    Methods: Using the UK-based Clinical Practice Research Datalink we identified 570 incident patients with GPA in 1990-2014, and for each case, we selected up to 10 random non-vasculitis patients matched for age, sex, practice and years of history before the date of first GPA diagnosis. We compared the distribution of 13 pre-existing comorbidities and estimated the risk of each incident comorbidity after the cohort entry between GPA and non-vasculitis patients. Read More

    Associations between genetic risk variants for kidney diseases and kidney disease etiology.
    Sci Rep 2017 Oct 24;7(1):13944. Epub 2017 Oct 24.
    Institute of Genetic Epidemiology, Medical Center - University of Freiburg, Faculty of Medicine, Freiburg, Germany.
    Chronic kidney disease (CKD) is a global health problem with a genetic component. Genome-wide association studies have identified variants associated with specific CKD etiologies, but their genetic overlap has not been well studied. This study examined SNP associations across different CKD etiologies and CKD stages using data from 5,034 CKD patients of the German Chronic Kidney Disease study. 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

    Relapsing granulomatosis with polyangiitis with severe lung and upper respiratory tract involvement successfully treated with rituximab.
    Reumatologia 2017 31;55(4):208-212. Epub 2017 Aug 31.
    Department of Interial Diseases, Connective Tissue Diseases and Geriatrics, University of Gdansk, Gdansk, Poland.
    Granulomatosis with polyangiitis (GPA) is a chronic, relapsing, primary systemic small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies. The disease is characterised by the involvement of various organs. The relapse rate rises from about 20% at 12 months to about 60% at 5 years. Read More

    Case 32-2017. A 64-Year-Old Man with Dyspnea, Wheezing, Headache, Cough, and Night Sweats.
    N Engl J Med 2017 10;377(16):1569-1578
    From the Departments of Medicine (K.P., A.N., M.J.K., F.B.C.) and Radiology (F.J.F.), Massachusetts General Hospital, and the Departments of Medicine (K.P., A.N., M.J.K., F.B.C.) and Radiology (F.J.F.), Harvard Medical School - both in Boston.

    Heart conduction system defects and sustained ventricular tachycardia complications in a patient with granulomatosis with polyangiitis. A case report and literature review.
    Rev Bras Ter Intensiva 2017 Jul-Sep;29(3):386-390
    Hospital São Rafael - Salvador (BA), Brasil.
    Granulomatosis with polyangiitis is a rare systemic inflammatory disorder characterized by vasculitis of the small arteries, the arterioles and the capillaries together with necrotizing granulomatous lesions. This case reports on a young female patient, previously diagnosed with granulomatosis with polyangiitis, who was admitted to the intensive care unit with seizures and hemodynamic instability due to a complete atrioventricular heart block. The event was associated with multiple episodes of sustained ventricular tachycardia without any structural heart changes or electrolyte disturbances. Read More

    Angina in granulomatosis with polyangiitis: A case report.
    Exp Ther Med 2017 Oct 16;14(4):3569-3572. Epub 2017 Aug 16.
    Department of Cardiology, The Second Affiliated Hospital of Dalian Medical University, Dalian, Liaoning 116023, P.R. China.
    Granulomatosis with polyangiitis (GPA) often causes small blood vessel lesions in multiple organs, with less impact on large vessels. We report a case involving not only large blood vessels, but also angina. The computed tomographic angiography of large vessels and vascular ultrasonography of the extremity also suggested great arteries of the body were involved. Read More

    Necrobiotic xanthogranuloma scleritis in a case of granulomatosis with polyangiitis (Wegener's granulomatosis).
    Int Med Case Rep J 2017 3;10:323-328. Epub 2017 Oct 3.
    Department of Medicine, Rheumatology Division, Phramongkutklao Hospital, Phramongkutklao College of Medicine, Bangkok, Thailand.
    The purpose of this study was to describe a case of necrobiotic xanthogranuloma scleritis in a 53-year-old male with unilateral progressive visual loss, scleritis, prolonged fever, and multiple mononeuropathy. Scleral biopsy showed necrosis with small abscess, and the pathological tissues revealed submucosal infiltration of mononucleated foamy histiocytes (xanthoma cells), hemosiderin-laden macrophages, neutrophils, lymphocytes, plasma cells, and erythrocytes without Touton giant cells or cholesterol clefts. Serum protein electrophoresis showed polyclonal gammopathy. Read More

    Autoimmune comorbidity in chronic spontaneous urticaria: A systematic review.
    Autoimmun Rev 2017 Oct 14. Epub 2017 Oct 14.
    Charité - Universitätsmedizin Berlin, Department of Dermatology and Allergology, Charitéplatz 1, Berlin D-10117, Germany. Electronic address:
    Background And Objective: Numerous autoimmune diseases (AIDs) have been linked to chronic spontaneous urticaria (CSU). Here, we provide the first extensive and comprehensive evaluation of the prevalence of AIDs in patients with CSU and vice versa.

    Methods: A Pubmed and Google Scholar search was performed to identify studies reporting the prevalence of various AIDs in CSU and vice versa published before April 2017. Read More

    Disseminated histoplamosis in adolescent mimicking granulomatosis with polyangiitis.
    Rev Bras Reumatol Engl Ed 2017 Sep - Oct;57(5):479-482. Epub 2015 Jul 14.
    Universidade de São Paulo, Faculdade de Medicina, Unidade de Reumatologia Pediátrica, São Paulo, Brazil; Universidade de São Paulo, Faculdade de Medicina, Divisão de Reumatologia, São Paulo, Brazil. Electronic address:

    Granulomatosis with polyangiitis can cause periaortitis and pericarditis.
    Clin Case Rep 2017 Oct 11;5(10):1732-1733. Epub 2017 Sep 11.
    Department of Diagnostic RadiologyFaculty of Life SciencesKumamoto University1-1-1 HonjyoChuo-ku, Kumamoto860-8556Japan.
    Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous inflammation usually involving the upper and lower respiratory tracts and necrotizing vasculitis predominantly affecting small-sized vessels. Although categorized as a small vessel vasculitis, GPA can cause periaortitis and pericarditis. Physicians should recognize all categories of vasculitis can affect any size arteries. 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

    Bilateral Frontal Lobe Vasogenic Edema Resulting from Hypertrophic Pachymeningitis due to Granulomatosis with Polyangiitis.
    Intern Med 2017 Oct 11. Epub 2017 Oct 11.
    Department of Neurology, JA Toride Medical Center, Japan.
    A 61-year-old woman presented with a 1-month history of decreased activities of daily living. Magnetic resonance imaging revealed abnormal intensities of the bilateral frontal lobes and enhancement of the thickened dura matter. A biopsy of the dura mater revealed multinucleated giant cells. Read More

    Kv1.3 Channel Blockade Modulates the Effector Function of B Cells in Granulomatosis with Polyangiitis.
    Front Immunol 2017 26;8:1205. Epub 2017 Sep 26.
    Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands.
    B cells are central to the pathogenesis of granulomatosis with polyangiitis (GPA), exhibiting both (auto)antibody-dependent and -independent properties. Class-switched memory B cells in particular are a major source of pathogenic autoantibodies. These cells are characterized by high expression levels of Kv1. 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

    Prognostic significance of cavitary lung nodules in granulomatosis with polyangiitis - A clinical and imaging study of 225 patients.
    Arthritis Care Res (Hoboken) 2017 Oct 9. Epub 2017 Oct 9.
    Vasculitis clinic, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada.
    Background: Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis with pulmonary nodules as a common manifestation. Our study examined whether pulmonary nodules, and nodule type (solid versus cavitary), are associated with different disease manifestations and outcomes.

    Methods: Demographic, clinical, biological, radiological data at diagnosis, during follow-up, and treatments of GPA patients followed at the Mount Sinai Hospital (Canada) vasculitis clinic were analyzed. Read More

    Orbital Inflammation as a Presenting Sign for CREST Syndrome.
    Ophthal Plast Reconstr Surg 2017 Sep 27. Epub 2017 Sep 27.
    *Orbital Service, Moorfields Eye Hospital, and †Rheumatology Department, Guy's and St Thomas; Hospital, London, United Kingdom.
    A 61-year-old male was referred with a week's history of a painful and swollen left eye. Examination revealed normal visual acuities, left proptosis and global restriction of ocular ductions, and subretinal fluid at the macula. CT imaging confirmed thickening of the posterior scleral coat, with an associated choroidal effusion. Read More

    Echocardiographic features in patients with ANCA-associated vasculitis within 3 months before and after diagnosis.
    Clin Rheumatol 2017 Dec 8;36(12):2751-2759. Epub 2017 Oct 8.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.
    We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. Read More

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

    Long term azathioprine maintenance therapy in ANCA-associated vasculitis: combined results of long-term follow-up data.
    Rheumatology (Oxford) 2017 Nov;56(11):1894-1901
    Department of Internal Medicine and Nephrology, University Medical Center Groningen, Groningen, the Netherlands.
    Objective: We studied whether in ANCA-associated vasculitis patients, duration of AZA maintenance influenced relapse rate during long-term follow-up.

    Methods: Three hundred and eighty newly diagnosed ANCA-associated vasculitis patients from six European multicentre studies treated with AZA maintenance were included; 58% were male, median age at diagnosis 59.4 years (interquartile range: 48. Read More

    Gene Expression Profiling and Heterogeneity of Nonspecific Orbital Inflammation Affecting the Lacrimal Gland.
    JAMA Ophthalmol 2017 Nov;135(11):1156-1162
    Casey Eye Institute, Oregon Health & Science University, Portland.
    Importance: Although a variety of well-characterized diseases, such as sarcoidosis and granulomatosis with polyangiitis, affect the lacrimal gland, many patients with dacryoadenitis are diagnosed as having nonspecific orbital inflammation (NSOI) on the basis of histology and systemic disease evaluation. The ability to further classify the disease in these patients should facilitate selection of effective therapies.

    Objective: To test the a priori hypothesis that gene expression profiles would complement clinical and histopathologic evaluations in identifying well-characterized diseases and in subdividing NSOI into clinically relevant groups. Read More

    Targeted proteomics reveals promising biomarkers of disease activity and organ involvement in antineutrophil cytoplasmic antibody-associated vasculitis.
    Arthritis Res Ther 2017 Sep 29;19(1):218. Epub 2017 Sep 29.
    Department of Hematology, Clinical Immunology, and Infectious Diseases, Ehime University Graduate School of Medicine, Toon, Ehime, 791-0295, Japan.
    Background: Targeted proteomics, which involves quantitative analysis of targeted proteins using selected reaction monitoring (SRM) mass spectrometry, has emerged as a new methodology for discovery of clinical biomarkers. In this study, we used targeted serum proteomics to identify circulating biomarkers for prediction of disease activity and organ involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

    Methods: A large-scale SRM assay targeting 135 biomarker candidates was established using a triple-quadrupole mass spectrometer coupled with nanoflow liquid chromatography. Read More

    Alterations in circulating lymphoid cell populations in systemic small vessel vasculitis are non-specific manifestations of renal injury.
    Clin Exp Immunol 2017 Sep 28. Epub 2017 Sep 28.
    Trinity Health Kidney Centre, Trinity Translational Medicine Institute, Dublin, Ireland.
    Innate lymphocyte populations, such as innate lymphoid cells (ILCs), γδ T cells, invariant natural killer T (iNK T) cells and mucosal-associated invariant T (MAIT) cells are emerging as important effectors of innate immunity and are involved in various inflammatory and autoimmune diseases. The aim of this study was to assess the frequencies and absolute numbers of innate lymphocytes as well as conventional lymphocytes and monocytes in peripheral blood from a cohort of anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV) patients. Thirty-eight AAV patients and 24 healthy and disease controls were included in the study. Read More

    Validation of the EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis by disease content experts.
    RMD Open 2017 15;3(1):e000449. Epub 2017 Jun 15.
    Department of Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK.
    The European League Against Rheumatism recommendations for the management of antineutrophil cytoplasmic antibody-associated vasculitis have been recently published. Unique to recommendation development, they were also voted on by members of a learned society. This paper explores the wider validity of the recommendations among people who self-identify as clinicians caring for patients with vasculitis. Read More

    Long-term patient survival in a Swedish population-based cohort of patients with ANCA-associated vasculitis.
    RMD Open 2017 13;3(1):e000435. Epub 2017 Jul 13.
    Department of Laboratory Medicine, Division of Clinical Chemistry and Pharmacology, Lund University, Lund, Sweden.
    Objectives: Patients with antineutrophil cytoplasmatic antibodies-associated vasculitides (AAV) exhibit higher mortality than the general population. In the current study, we assessed whether cluster affiliation based on clinical presentation might predict mortality.

    Methods: With case record review, the outcomes for a population-based cohort of patients diagnosed with AAV in southern Sweden (catchment area of 0. Read More

    Eosinophilic granulomatosis with polyangiitis - new definition, new treatment? - comment of the article by Puéchal et al.
    Arthritis Rheumatol 2017 Sep 26. Epub 2017 Sep 26.
    Sechenov First Moscow State Medical University, Moscow, Russia.
    The recently published CHUSPAN 2 study addresses an important issue of the optimum treatment for nonsevere systemic necrotising vasculitis (SNV) that poses no immediate threat to life or essential organs function (1). This study was conducted by the French Vasculitis Study Group that contributed a lot to our understanding of systemic vasculitis. All SNVs have serious prognosis and require prompt immunosuppressive therapy. Read More

    Eosinophilic granulomatosis with polyangiitis - toward personalized treatment.
    Arthritis Rheumatol 2017 Sep 26. Epub 2017 Sep 26.
    Vasculitis Study Group, Cochin Hospital, Paris-Descartes University, Paris, France.
    Dr. Smitienko and colleagues commented on our trial on the interest of azathioprine (AZA) adjunction to glucocorticoids to induce remission of systemic necrotizing vasculitides (SNVs) without poor-prognosis factors, more specifically on eosinophilic granulomatosis with polyangiitis (EGPA). We fully agree that EGPA is a heterogenous disease. Read More

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