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    Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Hepatitis B Infection - A Rare Association.
    J Clin Diagn Res 2016 Dec 1;10(12):OD07-OD09. Epub 2016 Dec 1.
    Senior Resident, Department of Medicine, Himalayan Institute of Medical Sciences , Dehradun, Uttarakhand, India .
    Eosinophilic Granulomatosis with Polyangiitis (EGPA) or Churg-Strauss syndrome is a rare systemic illness which is characterized by necrotizing inflammation of small and medium sized vessels. The prominent features include asthma, eosinophilia, transient pulmonary infiltration and systemic vasculitis. Various triggering factors have been reported as putative aetiological agents of Churg-Strauss syndrome. Read More

    Anti-neutrophil cytoplasmic antibody-associated vasculitis with renal involvement: Analysis of 89 cases.
    Med Clin (Barc) 2017 Jan 27;148(1):1-7. Epub 2016 Oct 27.
    Servicio de Nefrología, Hospital Universitario Ramón y Cajal, Madrid, España.
    Introduction: The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with renal involvement are associated with high morbi-mortality. In this study we analyse if the prognosis of these diseases have improved in recent years, and which factors influence the outcomes.

    Material And Methods: Retrospective single-centre observational study, which included all patients diagnosed with microscopic polyangiitis and granulomatosis with polyangiitis with renal involvement in the last 25 years. Read More

    Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases.
    Int J Cardiol 2017 Feb 1. Epub 2017 Feb 1.
    Onassis Cardiac Surgery Center, Athens, Greece.
    Background-aim: Cardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis.

    Patients-methods: CMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Read More

    Rituximab therapy in pemphigus and other autoantibody-mediated diseases.
    F1000Res 2017 27;6:83. Epub 2017 Jan 27.
    Department of Dermatology, University of Pennsylvania, 1009 Biomedical Research Building, 421 Curie Boulevard, PA, USA.
    Rituximab, a monoclonal antibody targeting the B cell marker CD20, was initially approved in 1997 by the United States Food and Drug Administration (FDA) for the treatment of non-Hodgkin lymphoma. Since that time, rituximab has been FDA-approved for rheumatoid arthritis and vasculitides, such as granulomatosis with polyangiitis and microscopic polyangiitis. Additionally, rituximab has been used off-label in the treatment of numerous other autoimmune diseases, with notable success in pemphigus, an autoantibody-mediated skin blistering disease. Read More

    The forgotten disease - Lemierres syndrome.
    J Pak Med Assoc 2016 Dec;66(12):1652-1655
    Pakistan Institute of Medical Sciences, Islamabad.
    Lemierre's syndrome refers to septic thrombosis of deep veins of the neck, is a rare and often life threatening complication following upper respiratory tract infections. We present here a case of Lemierre's syndrome in a previously healthy 30 years old female who had a febrile illness for two weeks with associated dysphagia, hoarse voice and right sided neck swelling. She was investigated for retropharyngeal and parapharyngeal abscess, granulomatosis with polyangiitis, tuberculosis and thyroiditis but finally concluded as Lemierre's syndrome based on the findings of thrombosis of the deep neck veins following respiratory tract infection, septic pulmonary emboli and clinical recovery with antibiotics and supportive care. Read More

    Galactosylation and Sialylation Levels of IgG Predict Relapse in Patients With PR3-ANCA Associated Vasculitis.
    EBioMedicine 2017 Jan 28. Epub 2017 Jan 28.
    Center for Proteomics and Metabolomics, Leiden University Medical Center, Leiden, The Netherlands.
    Objective: The objective of our study is to investigate the Fc glycosylation profiles of both antigen-specific IgG targeted against proteinase 3 (PR3-ANCA) and total IgG as prognostic markers of relapse in patients with Granulomatosis with Polyangiitis (GPA).

    Methods: Seventy-five patients with GPA and a PR3-ANCA rise during follow-up were included, of whom 43 patients relapsed within a median period of 8 (2-16) months. The N-glycan at Asn297 of affinity-purified and denatured total IgG and PR3-ANCA was determined by mass spectrometry of glycopeptides in samples obtained at the time of the PR3-ANCA rise and at the time of the relapse or time-matched during remission. Read More

    Acute urinary retention secondary to urethral involvement of granulomatosis with polyangiitis.
    Can Urol Assoc J 2017 Jan-Feb;11(1-2):E38-E40. Epub 2017 Jan 12.
    Department of Surgery, Division of Urology; Schulich School of Medicine and Dentistry, Western University, London Health Sciences Centre, London, ON, Canada.
    Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a systemic necrotizing vasculitis of small- and medium-sized blood vessels, primarily affecting the upper and lower respiratory tracts, as well as the kidneys. Urogenital manifestations of GPA are exceedingly rare and usually respond well to systemic immunosuppressive therapy. Here, we present a case of a 36-year-old female presenting with acute urinary obstruction secondary to urethral GPA involvement in the immediate postpartum period. Read More

    Eosinophilic endomyocardial disease.
    Cardiovasc Pathol 2017 Jan 23;27:54-56. Epub 2017 Jan 23.
    Department of Internal Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, 6431 Fannin, MSB 1.150, Houston, TXs 77030, United States.
    Eosinophilic granulomatosis with polyangiitis is the rarest of the antineutrophil cytoplasmic antibody-associated vasculitides. We describe the case of a 58-year-old woman with a history of asthma found to have a left ventricular apical thrombus. Cardiac catheterization with endomyocardial biopsy was consistent with eosinophilic endomyocardial disease. Read More

    Skewing towards Treg and Th2 responses is a characteristic feature of sustained remission in ANCA-positive granulomatosis with polyangiitis.
    Eur J Immunol 2017 Feb 2. Epub 2017 Feb 2.
    Department of Medicine, Jagiellonian University Medical College, Krakow, Poland.
    The objective of our study was to evaluate the T-helper (Th) and regulatory T (Treg) cell profile in ANCA-positive granulomatosis with polyangiitis (GPA) and its relation to disease activity. In a prospective study, we studied two groups of GPA patients: (1) disease flare (active-GPA, BVAS>6, n = 19), (2) sustained remission (≥ 1-year prior enrollment, inactive-GPA, BVAS = 0, n = 18). 24 age-sex matched healthy subjects served as controls. Read More

    Rituximab in ANCA-Associated Vasculitis.
    Curr Rheumatol Rep 2017 Feb;19(2)
    Birmingham VA Medical Center, 700 19th St S., Birmingham, AL, 35233, USA.
    Purpose Of Review: The purpose of this review is to describe the efficacy and safety of rituximab (RTX) as a remission induction and maintenance therapy in ANCA-associated vasculitis (AAV).

    Recent Findings: A PubMed search was carried out to track down articles published between February 2006 and February 2016. Randomized controlled trials (RCTs) that encompassed patients with AAV were included. Read More

    Heart failure not responsive to standard immunosuppressive therapy is successfully treated with high dose intravenous immunoglobulin therapy in a patient with Eosinophilic Granulomatosis with Polyangiitis (EGPA).
    Int Immunopharmacol 2017 Jan 30;45:13-15. Epub 2017 Jan 30.
    Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy.
    Glucocorticoids and immunosuppressive drugs represent the first-line treatment of eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss syndrome), even though the combined therapy is not successful in achieving the disease remission in some patients with neurological or cardiac involvement. We describe a case of an EGPA male patient with impaired left ventricular function not responsive to glucocorticoid and immunosuppressive therapy. We observed that high-dose (2g/kg/4weeks) intravenous immunoglobulin (IVIG) therapy significantly improved cardiac function, which was deteriorated after reducing IVIG dose at 0. Read More

    ANCA-associated vasculitis.
    Clin Med (Lond) 2017 Feb;17(1):60-64
    Ipswich Hospital and honorary senior lecturer Norwich Medical School, University of East Anglia, Norwich, UK.
    The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome). Read More

    Pneumocystis jiroveci pneumonia in rheumatic disease: a 20-year single-centre experience.
    Clin Exp Rheumatol 2017 Jan 27. Epub 2017 Jan 27.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, USA.
    Objectives: Pneumocystis jiroveci pneumonia (PJP) is an opportunistic infection with high mortality among patients with underlying rheumatologic conditions. Given the paucity of prospective data to guide treatment, clinical guidelines to initiate PJP prophylaxis are based on expert opinion and identify patients on ≥20 mg daily prednisone for ≥4 weeks duration for treatment. Herein we describe the PJP experience in rheumatic disease over a 20-year period at a single academic medical centre to investigate this 20 mg threshold and risk associated with lymphocyte counts, co-existing lung disease and immunosuppressive medications. Read More

    [Antineutrophil cytoplasmic antibodies associated with infective endocarditis: Literature review].
    Rev Med Interne 2017 Jan 25. Epub 2017 Jan 25.
    Département de médecine interne, CHU de Rouen, 1, rue de Germont, 76031 Rouen cedex, France. Electronic address:
    Antineutrophil cytoplasmic antibodies (ANCA) associated with infective endocarditis are a rare disorder. The condition can mimic primary systemic vasculitis (i.e. Read More

    The Use of Integra® Dermal Regeneration Template for the Orbital Exenteration Socket: A Novel Technique.
    Ophthal Plast Reconstr Surg 2017 Jan 23. Epub 2017 Jan 23.
    Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan, U.S.A.
    Purpose: Integra® dermal regeneration template is a bilayer membrane system that acts as a scaffold for regenerating dermal skin cells. It is used for wound reconstruction following burns, extensive injuries, and a large tumor excision in multiple parts of the body. The dermal layer is made of porous matrix of bovine tendon collagen and glycosaminoglycan. Read More

    A pragmatic approach to vasculitis in the gastrointestinal tract.
    J Clin Pathol 2017 Jan 24. Epub 2017 Jan 24.
    Department of Pathology, Laboratory Medicine Program, University Health Network and University of Toronto, Toronto, Ontario, Canada.
    Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. Read More

    Autoimmune hepatitis and eosinophilic granulomatosis with polyangiitis: a rare association.
    BMJ Case Rep 2017 Jan 20;2017. Epub 2017 Jan 20.
    Department of Internal Medicine, Reading Health System, Sixth Avenue and Spruce Street, West Reading, Pennsylvania, USA.
    We describe a case of 40-year-old woman who presented to our hospital with symptoms of non-radiating epigastric pain for 4-5 days associated with nausea and vomiting. Her history was significant for asthma with recurrent exacerbations, polyneuropathy and recurrent sinus infections. Liver function tests revealed cholestasis. Read More

    Extracorporeal membrane oxygenation rescue in adolescent with bronchiolitis obliterans-organizing pneumonia like Wegener's granulomatosis.
    Clin Case Rep 2017 Jan 17;5(1):29-34. Epub 2016 Dec 17.
    ECMO Centre Karolinska Department of Pediatric Perioperative Medicine and Intensive Care Karolinska University Hospital Stockholm Sweden.
    We report a 17-year-old woman with bronchiolitis obliterans-organizing pneumonia (BOOP)-like granulomatosis with polyangiitis developing severe airway obliterations. Pending age, phase and grade of autoimmune treatment, and offering ECMO treatment may be crucial for survival but occasionally preface futility. ECMO-treated patient with BOOP-like GPA has never been described before. Read More

    Incidence of Malignancy Prior to Antineutrophil Cytoplasmic Antibody-associated Vasculitis Compared to the General Population.
    J Rheumatol 2017 Jan 15. Epub 2017 Jan 15.
    From the Department of Pathology, and the Department of Medical Statistics and Bioinformatics, Leiden University Medical Center, Leiden, the Netherlands. E.E. van Daalen is supported by the Kolff Student researcher grant from the Dutch Kidney Foundation. Part of this work was presented during the American Society of Nephrology Kidney Week 2015, for which van Daalen received a grant from the Koninklijke Nederlandse Akademie van Wetenschappen and from the Dutch Vasculitis Foundation. E.E. van Daalen, BSc, Department of Pathology, Leiden University Medical Center; C. Rahmattulla, BSc, Department of Pathology, Leiden University Medical Center; R. Wolterbeek, MD, Department of Medical Statistics and Bioinformatics, Leiden University Medical Center; J.A. Bruijn, MD, PhD, Department of Pathology, Leiden University Medical Center; and I.M. Bajema, MD, PhD, Department of Pathology, Leiden University Medical Center. Address correspondence to E.E. van Daalen, Department of Pathology, L1-Q (P0-107), Leiden University Medical Centre, P.O. Box 9600, 2300 RC Leiden, the Netherlands. E-mail: Accepted for publication November 10, 2016.
    Objective: Previous studies have reported an increased malignancy risk preceding antineutrophil cytoplasmic antibody-associated vasculitis (AAV), suggesting common pathogenic pathways in these 2 entities. However, the study results were conflicting and often limited to patients with granulomatosis with polyangiitis (GPA). Here, we study the malignancy risk prior to AAV diagnosis [either GPA or microscopic polyangiitis (MPA)] to elaborate on the putative association between malignancy and AAV. Read More

    Low Median Nerve Palsy as Initial Manifestation of Churg-Strauss Syndrome.
    J Hand Surg Am 2017 Jan 11. Epub 2017 Jan 11.
    Department of Orthopaedic Surgery, Seoul National University College of Medicine, Seoul, Korea.
    Anterior interosseous nerve (AIN) syndrome is typically characterized by forearm pain and partial or complete dysfunction of the AIN-innervated muscles. Although the exact etiology and pathophysiology of the disorder remain unclear, AIN syndrome is increasingly thought to be an inflammatory condition of the nerve rather than a compressive neuropathy because the symptoms often resolve spontaneously following prolonged observation. However, peripheral neuropathy can be 1 of the first symptoms of systemic vasculitis that needs early systemic immunotherapy to prevent extensive nerve damage. Read More

    Clinical trials in antineutrophil cytoplasmic antibody-associated vasculitis: what we have learnt so far, and what we still have to learn.
    Nephrol Dial Transplant 2017 Jan 12. Epub 2017 Jan 12.
    Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, MN, USA
    The prognosis of the antineutrophil cytoplasmic antibody associated vasculitides (AAV), microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), has been fundamentally improved over the last five decades by the use of glucocorticoids and immunosuppressants, turning them from consistently fatal diseases into chronic conditions. The long-term course is now largely determined by the frequency of disease flares and by accruing damage caused by disease activity and treatment-related comorbidities. This review summarizes the evidence derived from clinical trials performed during the last 30 years and the remaining clinical unmet needs that new studies aim to address. Read More

    Time-course of Serum Pro-inflammatory Cytokines and Chemokines Levels Observed in Granulomatosis with Polyangiitis: A Case Report.
    Sarcoidosis Vasc Diffuse Lung Dis 2016 Dec 23;33(4):407-412. Epub 2016 Dec 23.
    Nihon University School of Medicine.
    A 77-year-old man visited our hospital with chief complaints of difficulty in hearing, nasal discharge and fever. The patients was diagnosed with otitis media, and his fever continued at approximately 38°C despite the administration of clarithromycin. After that, dyspnea on exertion developed and chest X-ray examination indicated multiple infiltrative shadows. Read More

    Clinical Characteristics and Treatment Response of Peripheral Neuropathy in the Presence of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Experience at a Single Tertiary Center.
    J Clin Neurol 2017 Jan;13(1):77-83
    Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
    Background And Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA.

    Methods: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014. Read More

    Immunoglobulin (Ig)M antibodies to proteinase 3 in granulomatosis with polyangiitis and microscopic polyangiitis.
    Clin Exp Immunol 2017 Jan 11. Epub 2017 Jan 11.
    Mayo Clinic and Foundation, Rochester, MN, USA.
    Anti-neutrophil cytoplasmic antibodies (ANCA) appear to play an important role in the pathogenesis of ANCA-associated vasculitis (AAV). However, ANCA alone are not sufficient to generate disease, and some evidence suggests that infectious triggers may serve as inciting events for AAV disease activity. Antibodies of the immunoglobulin (Ig)M isotype often serve as markers of recent infection, and IgM ANCA have been identified previously in patients with AAV, although the frequency and clinical relevance of IgM ANCA is not well established. Read More

    The key clues to reach the diagnosis of Loeffler endomyocardial fibrosis associated with eosinophilic granulomatosis with polyangiitis.
    J Cardiovasc Med (Hagerstown) 2017 Jan 7. Epub 2017 Jan 7.
    a'De Gasperis' Cardio Center bRadiology Department Niguarda Hospital, Milan, Italy.
    Loeffler endomyocardial fibrosis is a rare restrictive cardiomyopathy due to chronic eosinophilic exposure (Loeffler endomyocarditis). Loeffler endomyocarditis is associated with different eosinophilic disorders, including eosinophilic granulomatosis with polyangiitis (EGPA). These images recapitulate all typical findings that can be observed in patients presenting with heart failure attributable to this specific cardiomyopathy associated with EGPA, a systemic medical condition that can be easily detectable just putting together different diagnostic elements. Read More

    Clinical Factors Associated with the Diagnosis of Granulomatosis with Polyangiitis.
    Otolaryngol Head Neck Surg 2017 Jan 1:194599816685696. Epub 2017 Jan 1.
    2 Division of Rheumatology, Department of Internal Medicine, School of Medicine, Izmir Katip Celebi University Ataturk Education and Research Hospital, Izmir, Turkey.
    Objective To determine the predictive value of nasal endoscopic findings and symptoms in the diagnosis of granulomatosis with polyangiitis (GPA). Study Design A cross-sectional study. Setting A tertiary university hospital. Read More

    Proteinase 3-antineutrophil cytoplasmic antibody-positive ulcerative colitis presenting with abducens neuropathy.
    BMJ Case Rep 2017 Jan 9;2017. Epub 2017 Jan 9.
    Department of Neurology, Seirei Hamamatu General Hospital, Hamamatsu, Shizuoka, Japan.
    A 72-year-old man with ulcerative colitis (UC) presented with complete left abducens nerve palsy. Although MRI showed no significant changes, cerebrospinal fluid analysis revealed pleocytosis and elevated protein and interleukin (IL)-6 levels. His serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) level was also elevated to 31. Read More

    Value of non-identified ANCA (non-PR3, non-MPO) in the diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis).
    Acta Clin Belg 2017 Jan 9:1-5. Epub 2017 Jan 9.
    a Department of Rheumatology , Hôpital Erasme, Université Libre de Bruxelles , Bruxelles , Belgium.
    Objective: Determine the frequency of granulomatosis with polyangiitis (GPA) associated with non-identified ANCA (non-MPO, non-PR3 ANCA) and secondarily compare their clinic with GPA associated with MPO-positive or PR3-positive ANCA.

    Methods: In a monocentric retrospective observational study, clinical data of 398 patients with non-identified ANCA (titer of ANCA at least 1/80 by immunofluorescence on ethanol fixed PMN) was gathered over a period of 6 years. GPA patients from this population were compared with GPA patients with identified ANCA on the basis of clinical, biological, immunological and histological features. Read More

    The Presence of Anti-Lactoferrin Antibodies in a Subgroup of Eosinophilic Granulomatosis with Polyangiitis Patients and Their Possible Contribution to Enhancement of Neutrophil Extracellular Trap Formation.
    Front Immunol 2016 23;7:636. Epub 2016 Dec 23.
    Department of Pathology, Hokkaido University Graduate School of Medicine , Sapporo , Japan.
    Lactoferrin (Lf) is one of the antigens of antineutrophil cytoplasmic antibodies (ANCA) and functions as an endogenous suppressor of neutrophil extracellular trap (NET) formation. However, the prevalence and pathogenicity of anti-lactoferrin antibodies (aLf) in ANCA-associated vasculitis (AAV) remain unrevealed. This study aimed to examine the significance of aLf in AAV, initially. Read More

    ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.
    J Stroke Cerebrovasc Dis 2017 Mar 5;26(3):e47-e49. Epub 2017 Jan 5.
    Stroke Unit, Mediterraneo Hospital, Athens, Greece.
    Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Read More

    Paediatric anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: an update on renal management.
    Pediatr Nephrol 2017 Jan 6. Epub 2017 Jan 6.
    Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
    The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening. Read More

    A Rare Right Ventricular Involvement of Eosinophilic Granulomatosis Demonstrated by Cardiac MRI.
    Pol J Radiol 2016 13;81:598-601. Epub 2016 Dec 13.
    Department of Clinical Imaging, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.
    Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis with a prevalence rate of seven per million. Cardiac involvement was reported in 20-50%, yet with improved diagnostic methods, the frequency of cardiac involvement is expected to be even higher. It can result in significant morbidity and mortality, accounting for about 50% of death. Read More

    [Granulomatosis with polyangiitis].
    Dtsch Med Wochenschr 2017 Jan 5;142(1):24-31. Epub 2017 Jan 5.
    Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Read More

    Gastrointestinal tract involvement in granulomatosis with polyangiitis.
    Prz Gastroenterol 2016 11;11(4):270-275. Epub 2016 Feb 11.
    Department and Clinic of Nephrology, Transplantology, and Internal Medicine, Medical University of Gdansk, Gdansk, Poland.
    Introduction: Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis of small arteries and veins. In its classical manifestation GPA affects the upper and lower respiratory tract and kidneys. However, other organs, including those of the gastrointestinal tract, may be affected as well. Read More

    Environmental factor and inflammation-driven alteration of the total peripheral T-cell compartment in granulomatosis with polyangiitis.
    J Autoimmun 2016 Dec 28. Epub 2016 Dec 28.
    Department of Rheumatology & Vasculitis Center UKSH, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany. Electronic address:
    Autoimmune diseases are initiated by a combination of predisposing genetic and environmental factors resulting in self-perpetuating chronic inflammation and tissue damage. Autoantibody production and an imbalance of effector and regulatory T-cells are hallmarks of autoimmune dysregulation. While expansion of circulating effector memory T-cells is linked to disease pathogenesis and progression, the causes driving alterations of the peripheral T-cell compartment have remained poorly understood so far. Read More

    Employment, work disability and quality of life in patients with ANCA-associated vasculitides. The EXPOVAS study.
    Clin Exp Rheumatol 2016 Dec 20. Epub 2016 Dec 20.
    Department of Internal Medicine, National Referral Centre for Rare Autoimmune and Systemic Diseases, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, France.
    Objectives: Improved therapeutic strategies for ANCA-associated vasculitis (AAV) have transformed acute and life-threatening diseases into chronic ones responsible for marked morbidity that could impact employment, work disability and quality of life (QoL). We aimed to analyse work, handicaps and QoL of AAV patients and identify their determinants.

    Methods: Patients with AAV were included in a cross-sectional study assessing employment, work disability and QoL. Read More

    Severe bilateral bronchial stenosis with acute respiratory failure from granulomatosis with polyangiitis.
    Respirol Case Rep 2016 Nov 20;4(6):e00189. Epub 2016 Sep 20.
    Department of Radiology, Faculty of Medicine Chiang Mai University Chiang Mai Thailand.
    We report a 48-year-old female patient hospitalized with dyspnoea, wheezing, and respiratory failure due to bilateral main bronchial stenosis from granulomatosis with polyangiitis (GPA) involvement. By computed tomography imaging and flexible bronchoscopy, we measured the narrowest diameter at 2 mm. The patient promptly recovered from respiratory failure after treatment with flexible bronchoscopic balloon dilatation (BBD) without any procedure-related adverse event. Read More

    Identification of functional and expression polymorphisms associated with risk for anti-neutrophil cytoplasmic autoantibody-associated vasculitis.
    Arthritis Rheumatol 2016 Dec 28. Epub 2016 Dec 28.
    Mount Sinai Hospital, Lunenfeld-Tanenbaum Research Institute and Toronto General Research Institute, Toronto, ON, Canada.
    Objective: To identify risk alleles relevant to the cause and biology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

    Methods: We conducted a genome-wide association and subsequent replication study including 1986 cases of AAV [granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)] and 4723 controls. Meta-analysis of these datasets and functional annotation of identified risk loci were performed and candidate disease variants with unknown functional effects investigated for impact on gene expression and/or protein function. Read More

    Granulomatosis with polyangiitis confined to lacrimal gland, a case report.
    Arch Soc Esp Oftalmol 2016 Dec 22. Epub 2016 Dec 22.
    Instituto de Oftalmología «Fundación de Asistencia Privada Conde de Valenciana IAP», Ciudad de México, México.
    Clinical Case: A 43 year-old woman consulted due to 2 months of swelling on the superolateral side of the left orbit, with pain and erythema. An excisional biopsy was performed that revealed vasculitis with polyangiitis of the lacrimal gland. A systemic study showed that no other system was compromised. Read More

    The incidence, prevalence and mortality of granulomatosis with polyangiitis in the UK Clinical Practice Research Datalink.
    Rheumatology (Oxford) 2016 Dec 24. Epub 2016 Dec 24.
    Division of Epidemiology and Public Health, University of Nottingham.
    Objective: To estimate the incidence, prevalence and mortality of granulomatosis with polyangiitis in the UK.

    Methods: We conducted a historical cohort study using data from the Clinical Practice Research Datalink and Hospital Episode Statistics (HES). We calculated incidence rate ratios, adjusted for age, gender and ethnicity, using Poisson regression. Read More

    Pachymeningitis in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review.
    Clin Rheumatol 2016 Dec 23. Epub 2016 Dec 23.
    Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Calzada de Tlalpan 4502, Col. Sección XVI, Tlalpan, C.P, 14080, Mexico City, Mexico.
    The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Read More

    Adaptive Optics Imaging in Retinal Vasculitis.
    Ocul Immunol Inflamm 2016 Dec 23:1-7. Epub 2016 Dec 23.
    c Department of Cornea and Refractive Surgery , Narayana Nethralaya , Bangalore , India.
    Purpose: To study the sheathing of retinal vasculitis in various systemic autoimmune diseases using adaptive optics imaging (AOI).

    Methods: Prospective, observational case series with six patients: Behçet disease (n = 1); systemic lupus erythematosus (n = 1); idiopathic retinal vasculitis (n = 2); granulomatosis with polyangiitis (n = 1); and Takayasu aorta arteritis (n = 1). Fundus photograph (FP), fundus fluorescein angiography (FFA) were done in all cases at presentation. Read More

    Outcomes of Endoscopic Dacryocystorhinostomy in Secondary Acquired Nasolacrimal Duct Obstruction: A Case-Control Study.
    Ophthal Plast Reconstr Surg 2016 Dec 16. Epub 2016 Dec 16.
    *Department of Ophthalmology, Division of Oculoplastic Surgery, and †Department of Otolaryngology - Head and Neck Surgery, University of Washington, Seattle, Washington, U.S.A.
    Introduction: Secondary acquired nasolacrimal duct obstruction (SANDO), where the obstruction is caused by a known process, is becoming more commonly treated by endoscopic dacryocystorhinostomy (endoDCR). This study evaluates outcomes of endoDCR for cases of SANDO in comparison to endoDCR outcomes treating primary acquired nasolacrimal duct obstruction (PANDO).

    Methods: All patients undergoing endoDCR from 2006 to 2015 at a tertiary referral center were reviewed for etiology of nasolacrimal duct obstruction (NLDO) and success of procedure. Read More

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