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    Glaucoma Surgery in Scleromalacia: Using Endoscopic Cyclophotocoagulation where Conventional Filtration Surgery or Angle Procedures are contraindicated.
    J Curr Glaucoma Pract 2017 May-Aug;11(2):73-75. Epub 2017 Aug 5.
    Consultant, Department of Ophthalmology, St Thomas' Hospital, London United Kingdom.
    Aim: To describe the surgical management of glaucoma in a patient with severe scleromalacia, and secondary angle closure.

    Introduction: The management of glaucoma with coexisting scleromalacia plus secondary angle closure is challenging as most commonly performed incisional glaucoma surgery as well as minimally invasive glaucoma surgery (MIGS), which targets the drainage angle are all contraindicated.

    Case Report: Medically refractory glaucoma in a 60-year-old male with a 30-year history of granulomatosis with polyangiitis resulting in extensive severe scleromalacia, cicatricial lower lid retraction with significant conjunctival exposure, and widespread synechial angle closure from chronic anterior uveitis was managed with combined phacoemulsification cataract surgery, and endoscopic cyclophotocoagulation (ECP). Read More

    Neurological Complications in Eosinophilic Granulomatosis with Polyangiitis (EGPA): The Roles of History and the Results of Physical Examinations in the Diagnosis of EGPA.
    Intern Med 2017 Sep 15. Epub 2017 Sep 15.
    Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Japan.
    Objective To investigate the clinical symptoms, the physical and neurological findings, and the clinical course of neurological complications in eosinophilic granulomatosis with polyangiitis (EGPA). Methods A retrospective chart review of EGPA cases managed by two referral hospitals was performed, with a focus on the neurological findings. The study analyzed the symptoms at the onset of EGPA and investigated their chronological relationship. Read More

    GP2013: A Rituximab Biosimilar.
    BioDrugs 2017 Sep 18. Epub 2017 Sep 18.
    Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.
    GP2013 is the second biosimilar of the reference monoclonal anti-CD20 antibody rituximab to be approved in the EU. 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. GP2013 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. Read More

    Successful treatment with hyperbaric oxygen therapy for pneumatosis cystoides intestinalis as a complication of granulomatosis with polyangiitis: a case report.
    J Med Case Rep 2017 Sep 17;11(1):263. Epub 2017 Sep 17.
    Department of Surgery, Yokosuka General Hospital Uwamachi, 2-36 Uwamachi, Yokosuka, Kanagawa, 238-8567, Japan.
    Background: Although gastrointestinal involvement in patients with granulomatosis with polyangiitis is uncommon, it is associated with mild to severe life-threatening complications. We present a case of pneumatosis cystoides intestinalis in a patient with granulomatosis with polyangiitis that was treated successfully with hyperbaric oxygen.

    Case Presentation: A 70-year-old Japanese man with a 3-year history of granulomatosis with polyangiitis consulted our hospital with a complaint of severe back pain. Read More

    Rituximab as Induction Therapy in Eosinophilic Granulomatosis with Polyangiitis Refractory to Conventional Immunosuppressive Treatment: A 36-Month Follow-Up Analysis.
    J Allergy Clin Immunol Pract 2017 Sep 12. Epub 2017 Sep 12.
    Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. Electronic address:
    Background: Rituximab (RTX) is approved for induction therapy of granulomatosis with polyangiitis and microscopic polyangiitis. In eosinophilic granulomatosis with polyangiitis (EGPA), organ-threatening manifestations are mainly treated with cyclophosphamide (CYC). RTX as treatment in EGPA has been described in small case series; however long-term data and the efficacy of RTX in EGPA refractory to CYC have not been reported yet. Read More

    [Scleritis and episcleritis].
    J Fr Ophtalmol 2017 Sep 12. Epub 2017 Sep 12.
    Service d'ophtalmologie, Nouvel hôpital civil, université de Strasbourg, hôpitaux universitaires, BP 426, 67091 Strasbourg, France.
    Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly granulomatosis with polyangiitis (Wegener's) and polychondritis, and less often inflammatory bowel disease and systemic lupus erythematosus. Read More

    Central nervous system involvement in patients with granulomatosis with polyangiitis: a single-center retrospective study.
    Clin Rheumatol 2017 Sep 15. Epub 2017 Sep 15.
    Department of Pathophysiology, National and Kapodistrian University of Athens, Faculty of Medicine, Athens, Greece.
    The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe the related clinical characteristics and long-term outcomes of these patients. Medical charts of all ANCA-associated vasculitis patients were retrospectively reviewed, and GPA patients with CNS involvement were identified. Demographics, serological, and clinical features throughout the disease course were recorded. Read More

    Granulomatosis with Polyangiitis with Myocarditis and Ventricular Tachycardia.
    Case Rep Med 2017 20;2017:6501738. Epub 2017 Aug 20.
    Surgical Consultative Nephrology, UCLA Stone Center, Los Angeles, CA, USA.
    Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a pulmonary-renal syndrome affecting small and medium sized blood vessels. The disease has a prevalence in studies ranging from 3 to 15.7 cases per 100,000, with a noted increasing incidence and prevalence in more recent studies. Read More

    Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis.
    Nat Rev Rheumatol 2017 Sep 14. Epub 2017 Sep 14.
    Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Centre Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Eugenstrasse 3, 73230 Kirchheim unter Teck, Germany.
    Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications. Read More

    Unmet Needs in the Pathogenesis and Treatment of Vasculitides.
    Clin Rev Allergy Immunol 2017 Sep 11. Epub 2017 Sep 11.
    Division of Rheumatology, Department of Medicine, Arcispedale Santa Maria Nuova Istituto di Ricovero e Cura a Carattere Scientifico, Viale Risorgimento, 80, 42123, Reggio Emilia, Italy.
    Despite the progress in the last years on the field of vasculitides, there are several unmet needs regarding classification, disease activity assessment, predictors of flares and complications, and type of treatment for the different forms. The 1990 American College of Rheumatology (ACR) classification criteria currently used to define giant cell arteritis and Takayasu arteritis were designed to discriminate between different types of vasculitides but not to differentiate vasculitis from other disorders. Recently, efforts have been made to overcome the shortcomings of the ACR criteria. Read More

    Peripheral Arterial Tonometry in Pulmonary Vasculitis.
    Adv Exp Med Biol 2017 Sep 10. Epub 2017 Sep 10.
    Department of Family Medicine, Internal and Metabolic Diseases, Warsaw Medical University, and Systemic Vasculitis Outpatient Clinic Czerniakowski Hospital, Warsaw, Poland.
    Vascular wall inflammation in primary vasculitides results in diminished vessel dilation and finally impaired blood flow, causing multiple organs dysfunction and ultimate damage. In granulomatosis with polyangiitis (GPA), the inflammatory process concerns small and medium sized vessels and its pulmonary location is often predominant. The pivotal role in the development of that pathology plays vascular endothelium. Read More

    Granulomatosis with Polyangiitis Presenting with Pyoderma Gangrenosum-Like Ulceration and Negative Cytoplasmic Antineutrophilic Cytoplasmic Antibodies in a Child.
    Pediatr Dermatol 2017 Sep;34(5):e231-e234
    Geisinger Health System, Danville, Pennsylvania.
    Granulomatosis with polyangiitis (GPA), a vasculitis that most commonly affects small to medium-size vessels of the respiratory tract and kidneys, may also present with a wide array of skin findings. We present the case of a 12-year-old boy with pyoderma gangrenosum-like ulcerations on his lower extremity as the initial manifestation of GPA despite negative cytoplasmic antineutrophilic cytoplasmic antibodies (c-ANCAs). Although GPA is strongly associated with c-ANCA, c-ANCA may be negative on presentation. Read More

    Dacryoadenitis with Ptosis and Diplopia as the Initial Presentation of Granulomatosis with Polyangiitis.
    Intern Med 2017 Sep 6. Epub 2017 Sep 6.
    Department of Respiratory Medicine, Shonan Fujisawa Tokushukai Hospital, Japan.
    A 77-year-old Japanese woman presented to an ophthalmologist with an erythematous swollen upper eyelid and diplopia which was initially diagnosed to be idiopathic dacryoadenitis on the basis of a histological evaluation of an orbital mass that was in remission following a 3.5-month period of systemic corticosteroid therapy. She subsequently developed respiratory symptoms, and was finally diagnosed with systemic granulomatosis with polyangiitis (GPA) based on the clinical and histological features. Read More

    Successful Retrograde Intubation After Failed Fiberoptic Intubation and Percutaneous Cricothyrotomy.
    J Emerg Med 2017 Sep 4. Epub 2017 Sep 4.
    Department of Emergency Medicine, University of California-Los Angeles, Los Angeles, California.
    Background: An obstructive neck lesion presents an airway challenge for any emergency physician. Retrograde intubation is an infrequently used airway alternative that can be employed in the difficult airway algorithm that requires little training and is less invasive than surgical cricothyrotomy.

    Case Report: We report a case of a 31-year-old male patient who presented with respiratory distress progressing to respiratory failure from upper airway obstruction. Read More

    The "4 plus 2" rituximab protocol makes maintenance treatment unneeded in patients with refractory ANCA-associated vasculitis: A 10 years observation study.
    Oncotarget 2017 Aug 23;8(32):52072-52077. 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

    The epidemiology of ANCA associated vasculitis in Olmsted County, Minnesota (USA): a 20 year population-based study.
    Arthritis Rheumatol 2017 Sep 7. Epub 2017 Sep 7.
    Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN,.
    Objective: To estimate the incidence, prevalence and mortality of ANCA-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), in a U.S. based adult population. Read More

    Saddle nose deformity and septal perforation in granulomatosis with polyangiitis.
    Clin Otolaryngol 2017 Sep 7. Epub 2017 Sep 7.
    Department of Otorhinolaryngology, Head and Neck Surgery, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Campus Benjamin Franklin, Hindenburgdamm 30, 12200, Berlin, Germany.
    Background: Patients who have granulomatosis with polyangiitis (GPA, syn. M. Wegener) often develop an external nose deformity which may have devastating psychological effects. Read More

    Voriconazole-induced periostitis deformans: serial imaging in a patient with ANCA vasculitis.
    Skeletal Radiol 2017 Sep 2. Epub 2017 Sep 2.
    Rheumatology Department, Beaumont Hospital, Royal Oak, MI, USA.
    Objectives: A 61-year-old with acute granulomatosis and polyangiitis developed Aspergillus fumigatus pneumonia after admission to the intensive care unit with a small bowel perforation. This occurred after immunosuppression (intravenous methylprednisolone, intravenous cyclophosphamide, and plasmapheresis) for his initial presentation with stage 3 acute kidney injury.

    Materials And Methods: The mycologist recommended long-term treatment with voriconazole after initial recovery. Read More

    Demographic and Clinical Characteristics Associated with Central Nervous System Hemorrhage in Patients with Eosinophilic Granulomatosis with Polyangiitis: A Case Report and Review of the Literature.
    J Rheumatol 2017 Sep;44(9):1413-1415
    Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Virgen de las Nieves University Hospital, and Virgen de las Nieves University Hospital, Granada, Spain.

    Immunoadsorption in Autoimmune Diseases Affecting the Kidney.
    Semin Nephrol 2017 Sep;37(5):478-487
    Department of Nephrology, Medical University of Vienna, Vienna, Austria.
    Autoantibodies play an important role in the pathophysiology of renal involvement in systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic vasculitis, and anti-glomerular basement membrane disease (or Goodpasture syndrome). Direct removal of autoantibodies therefore has been tried in various ways, first by plasma exchange. Today, immunoadsorption is the extracorporeal method that most effectively removes (pathogenic) immune complexes and antibodies. Read More

    Immunoglobulin levels and infection risk with rituximab induction for anti-neutrophil cytoplasmic antibody-associated vasculitis.
    Clin Kidney J 2017 Aug 12;10(4):470-474. Epub 2017 Apr 12.
    Department of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Background: Rituximab (RTX), a B cell-depleting anti-CD20 monoclonal antibody, is approved for treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Low immunoglobulin (Ig) levels have been observed surrounding RTX treatment. The association between the degree of Ig deficiency and infection risk is unclear in AAV patients. Read More

    Clinical characteristics and long-term survival differences of the ANCA-associated vasculitis group: a cross-sectional study of 27 patients.
    Acta Med Litu 2017 ;24(2):107-112
    Centre of Rheumatology, Vilnius University Hospital Santaros klinikos, Vilnius, Lithuania.
    Objective: The goal of this study was to describe long-term patient survival and possible prognostic factors of a group of patients diagnosed with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) hospitalized at the tertiary Rheumatology Centre in Vilnius.

    Material And Methods: A cross-sectional study of 27 patients hospitalized at the Rheumatology Centre of Santaros klinikos of Vilnius University Hospital from 1 January 2001 to 31 December 2015 with diagnoses of GPA and MPA were carried out. Data on demographics, clinical characteristics, laboratory data, and the Birmingham Vasculitis Activity Score were collected. Read More

    ANCA Glomerulonephritis and Vasculitis.
    Clin J Am Soc Nephrol 2017 Aug 25. Epub 2017 Aug 25.
    Department of Pathology and Laboratory Medicine, Department of Medicine, and Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
    ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). Read More

    Rare occupational cause of nasal septum perforation: Nickel exposure.
    Int J Occup Med Environ Health 2017 Aug 23. Epub 2017 Aug 23.
    Hacettepe University, Ankara, Turkey (Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology).
    Many etiologies are held accountable for nasal septum perforations. Topical nasal drug usage, previous surgeries, trauma, nose picking, squamous cell carcinoma, some rheumatological disorders such as granulomatosis with polyangiitis (Wegener granulomatosis), some infectious diseases such as syphilis and leprosy are among the causes of the perforations. Occupational heavy metal exposures by inhalation rarely may also cause nasal septum perforation. Read More

    Primitive Neuroectodermal Tumor and Wegener's Granulomatosis of the Kidney: A Curious Combination of Two Rare Entities.
    Case Rep Urol 2017 1;2017:1750694. Epub 2017 Aug 1.
    Department of Pathology, University of Washington, Seattle, WA, USA.
    Wegener's granulomatosis (WG) is characterized by necrotizing polyangiitis involving the respiratory tract and kidneys. It causes segmental necrotizing glomerulonephritis in the kidneys. In rare cases, a renal pseudotumor may be seen because of the granulomatous process. Read More

    The role of biopsy in lacrimal gland inflammation: A clinicopathologic study.
    Orbit 2017 Aug 17:1-8. Epub 2017 Aug 17.
    e Department of Radiology , University of British Columbia and the Vancouver General Hospital , Vancouver , BC , Canada.
    Purpose: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation.

    Methods: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i. Read More

    Granulomatosis with Polyangiitis Presenting as Pyrexia of Unknown Origin, Leukocytosis, and Microangiopathic Haemolytic Anemia.
    Case Rep Rheumatol 2017 24;2017:6484092. Epub 2017 Jul 24.
    Maimonides Medical Center, Brooklyn, NY, USA.
    A 66-year-old woman presented to the Emergency Department with a florid sepsis-like picture, a two-week history of fever, relative hypotension with end organ ischemia (unexplained liver enzyme and troponin elevations), and nonspecific constitutional symptoms. She was initially found to have a urinary tract infection but, despite appropriate treatment, her fever persisted and her white blood cell count continued to rise. During her hospitalization the patient manifested leukocytosis to 47,000 WBC/μL, ESR 67 mm/hr (normal range 0-42 mm/hr), CRP 17. Read More

    Differential diagnosis of granulomatous lung disease: clues and pitfalls: Number 4 in the Series "Pathology for the clinician" Edited by Peter Dorfmüller and Alberto Cavazza.
    Eur Respir Rev 2017 Sep 9;26(145). Epub 2017 Aug 9.
    Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany
    Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Read More

    Cavitary lung lesions: Melioidosis and pulmonary embolism causing necrotizing pneumonia.
    IDCases 2017 20;10:4-6. Epub 2017 Jul 20.
    Department of Internal Medicine, Health campus, Universiti Sains Malaysia, Kelantan, Malaysia.
    Cavitary lung lesions of various etiologies may be encountered in patients with respiratory symptoms associated with fever. Non-malignant cavitary lesions may mimic malignant lung lesions on most of radiographic modalities including chest radiographs or thoracic computed tomography (CT). Primary lung malignancy can be detected in as high as one-fifths of CT thorax as cavitary lesions and the remaining aetiologies may be due to bacterial, parasitic, and invasive fungal infections, as well as Granulomatosis with polyangiitis (GPA), sarcoidosis, septic thrombo-embolism, and lung metastasis from extra-pulmonary primaries. Read More

    Peripheral Ulcerative Keratitis Associated with Autoimmune Disease: Pathogenesis and Treatment.
    J Ophthalmol 2017 13;2017:7298026. Epub 2017 Jul 13.
    Department of Ophthalmology, China-Japan Union Hospital of Jilin University, Changchun City, China.
    Peripheral ulcerative keratitis (PUK) is type of crescent-shaped inflammatory damage that occurs in the limbal region of the cornea. PUK is always combined with an epithelial defect and the destruction of the peripheral corneal stroma. PUK may have a connection to systemic conditions, such as long-standing rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Wegener granulomatosis (WG), relapsing polychondritis, classic polyarteritis nodosa and its variants, microscopic polyangiitis, and Churg-Strauss syndrome. Read More

    Clinical practice variation and need for pediatric-specific treatment guidelines among rheumatologists caring for children with ANCA-associated vasculitis: an international clinician survey.
    Pediatr Rheumatol Online J 2017 Aug 7;15(1):61. Epub 2017 Aug 7.
    Clinical Professor, Division of Rheumatology, Department of Pediatrics, University of British Columbia, BC Children's Hospital, Room K4-119 4480 Oak Street Vancouver, Vancouver, BC, V6H 3V4, Canada.
    Background: Because pediatric antineutrophil cytoplasmic antibody-associated vasculitis is rare, management generally relies on adult data. We assessed treatment practices, uptake of existing clinical assessment tools, and interest in pediatric treatment protocols among rheumatologists caring for children with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

    Methods: A needs-assessment survey developed by an international working group of pediatric rheumatologists and two nephrologists was circulated internationally. Read More

    Left ventricular thrombectomy in myocarditis: the epicardial scan & video-assisted transaortic approach.
    Minim Invasive Ther Allied Technol 2017 Aug 8:1-4. Epub 2017 Aug 8.
    a Cardiovascular Department , University Hospital of Trieste , Trieste , Italy.
    In a 23-year-old man having myocarditis in the context of eosinophilic granulomatosis with polyangiitis, a mobile left ventricular apical thrombus was found with transthoracic echocardiography. Its surgical removal was established because there were no signs of resizing after effective intravascular anticoagulation therapy. Surgery was carried out via a median sternotomy with cardiopulmonary bypass. Read More

    Skin nodule reveals lung cancer in a patient with granulomatosis with polyangiitis.
    Clin Case Rep 2017 Aug 5;5(8):1339-1340. Epub 2017 Jul 5.
    Division of ImmunologyDepartment of Internal MedicineUniversity of Iowa Hospitals and ClinicsIowa CityIowa.
    A 69-year-old male with granulomatosis with polyangiitis presented with new skin nodules. Skin biopsy showed metastatic poorly differentiated lung adenocarcinoma. The skin nodule was the initial presentation of his lung cancer. Read More

    "How I treat" autoimmune diseases: State of the art on the management of rare rheumatic diseases and ANCA-associated systemic idiopathic vasculitis.
    Autoimmun Rev 2017 Oct 2;16(10):995-998. Epub 2017 Aug 2.
    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, and University of Turin, Italy; SCU Nephrology and Dialysis Unit, S. Giovanni Bosco Hospital and University of Turin, Turin, Italy. Electronic address:
    This Special Issue of Autoimmunity Reviews constitutes summaries of presentations at the 20th International Meeting on Immunopathology and Orphan Diseases, held in Torino, Italy, 25-28th January 2017. As such, these presentations represent the state of the art on the pathophysiology of autoimmune diseases as well as the most recent insights into the management of these pathologic conditions. The latter includes both the optimal use of established drugs and approaches as well as novel knowledge on the means and consequences of targeted blocking of molecules or cellular mechanisms. Read More

    Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.
    Autoimmun Rev 2017 Oct 2;16(10):1036-1043. Epub 2017 Aug 2.
    Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Université Paris Descartes, Paris 5, Paris, France. Electronic address:
    Objective: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap.

    Methods: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Read More

    Accuracy of Magnetic Resonance Imaging for Grading of Subglottic Stenosis in Patients with Granulomatosis with Polyangiitis: Correlation with Pulmonary Function Tests and Laryngoscopy.
    Arthritis Care Res (Hoboken) 2017 Aug 3. Epub 2017 Aug 3.
    Department of Diagnostic and Interventional Radiology and Nuclear Medicine, Center of Endoscopy and Radiology, University Hospital Hamburg-Eppendorf, Hamburg, Germany.
    Objectives: To compare magnetic resonance imaging (MRI)-based and laryngoscopy-based subglottic stenosis (SGS) grading with pulmonary function tests (PFT) in patients with granulomatosis with polyangiitis (GPA).

    Methods: In this retrospective study we included 118 examinations of 44 patients with GPA and suspected SGS. All patients underwent MRI, laryngoscopy, and PFT. Read More

    Prospective monitoring of in vitro produced PR3-ANCA does not improve relapse prediction in granulomatosis with polyangiitis.
    PLoS One 2017 3;12(8):e0182549. Epub 2017 Aug 3.
    Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.
    Objectives: Patients with granulomatosis with polyangiitis (GPA) are prone to disease relapse. Currently, no good biomarkers are available to predict relapses in individual patients. This study aimed to determine whether patients at risk for relapse can be distinguished based on increased in vitro autoantibody production. Read More

    Three atypical manifestations of granulomatosis with polyangiitis: lateral medullary syndrome, anterior cheek mass and melting scleritis of eye.
    Reumatologia 2017 18;55(3):145-150. Epub 2017 Jul 18.
    Clinical Neurology Research Center, Departments of Neurology, Shiraz University of Medical Sciences, Iran.
    Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a vasculitis with various organ involvement. There have been a few cases of CNS stroke and rare cases of lateral medullary infarction (LMI) as a manifestation of GPA. Also there have been reports of sinuses, nose and laryngeal masses mistakenly referred as carcinomas and subsequently GPA was diagnosed in their pathological reports. Read More

    Birmingham vasculitis activity score of more than 9.5 at diagnosis is an independent predictor of refractory disease in granulomatosis with polyangiitis.
    Int J Rheum Dis 2017 Aug 1. Epub 2017 Aug 1.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
    Aim: We investigated whether clinical manifestations, anti-neutrophil cytoplasmic antibodies (ANCAs), Birmingham vasculitis activity score (BVAS) for granulomatosis with polyangiitis (GPA) and five factor score (FFS) at diagnosis can predict relapse or refractory disease in 30 histology-proven GPA patients with follow-up duration ≥ 12 weeks.

    Methods: We reviewed the medical records of 30 GPA patients. We collected clinical data, ANCAs, BVAS for GPA, FFSs at diagnosis, and we compared variables between the two groups based on relapse or refractory disease. Read More

    Outcome of kidney transplantation in pediatric patients with ANCA-associated glomerulonephritis: a single-center experience.
    Pediatr Nephrol 2017 Aug 1. Epub 2017 Aug 1.
    Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
    Objectives: Data on kidney transplant outcomes for pediatric patients with end-stage renal disease (ESRD) secondary to anti-neutrophil cytoplasmic antibody glomerulonephritis (ANCA GN), particularly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), is limited. We describe our experience of kidney transplantation in pediatric ANCA GN patients.

    Methods: We performed a retrospective review of patients with ANCA GN who developed ESRD and were transplanted at a single center between the years 2000 and 2014. Read More

    Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype.
    J Rheumatol 2017 Aug 1. Epub 2017 Aug 1.
    From the Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden; Vasculitis and Lupus Clinic, and Department of Radiology, and Department of Respiratory Medicine, Addenbrooke's Hospital, Cambridge, UK. Supported by research grants from Governmental Funding of Clinical Research within the Faculty of Medicine, Lund University (ALF-medel), The Swedish Society of Medicine (Svenska Läkarsällskapet), and the UK National Institute for Health Research Cambridge Biomedical Research Centre. A.J. Mohammad, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University, and Vasculitis and Lupus Clinic, Addenbrooke's Hospital; K.H. Mortensen, MD, PhD, Department of Radiology, Addenbrooke's Hospital; J. Babar, MBChB, MRCP, FRCR, Department of Radiology, Addenbrooke's Hospital; R. Smith, MA, Vasculitis and Lupus Clinic, Addenbrooke's Hospital; R.B. Jones, MD, MRCP, Vasculitis and Lupus Clinic, Addenbrooke's Hospital; D. Nakagomi, MD, PhD, Vasculitis and Lupus Clinic, Addenbrooke's Hospital; P. Sivasothy, MBBS, PhD, Department of Respiratory Medicine, Addenbrooke's Hospital; D.R. Jayne, FMedSci, Vasculitis and Lupus Clinic, Addenbrooke's Hospital. Address correspondence to Dr. A.J. Mohammad, Department of Clinical Sciences, Rheumatology, Lund University, S-221 85, Lund, Sweden. E-mail: Accepted for publication May 17, 2017.
    Objective: To describe pulmonary involvement at time of diagnosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), as defined by computed tomography (CT).

    Methods: Patients with thoracic CT performed on or after the onset of AAV (n = 140; 75 women; granulomatosis with polyangiitis, n = 79; microscopic polyangiitis MPA, n = 61) followed at a tertiary referral center vasculitis clinic were studied. Radiological patterns of pulmonary involvement were evaluated from the CT studies using a predefined protocol, and compared to proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA specificity. Read More

    Sequential rituximab and omalizumab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
    Clin Rheumatol 2017 Jul 31. Epub 2017 Jul 31.
    GIRAT: Grupo de Investigación en Reumatología, Autoinmunidad y Medicina Traslacional, Fundación Valle del Lili and Univesidad Icesi, Cali, Colombia.
    Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Read More

    Tuberculosis, granulomatosis with polyangiitis, or both? A case report.
    Arch Soc Esp Oftalmol 2017 Jul 27. Epub 2017 Jul 27.
    Instituto de Oftalmología Fundación de Asistencia Privada Conde de Valenciana IAP, Ciudad de México, México.
    Introduction: Tuberculosis (TB) is a chronic granulomatose infection, and granulomatosis with polyangiitis (GP) is a small vessel vasculitis, both of which affect the lungs. The combination of these diseases is rare. Both have similar clinical features, making the differential diagnosis difficult. Read More

    Necrotizing sarcoid granulomatosis: A distinctive form of pulmonary granulomatous disease.
    Clin Respir J 2017 Jul 29. Epub 2017 Jul 29.
    Department of Pneumonology, Democritus University of Thrace, Alexandroupolis, Greece.
    Objectives: To define the characteristics of necrotizing sarcoid granulomatosis (NGS) a very rare pulmonary disease hardly recognised by pulmonologists and pathologists.

    Data Source: PubMed was searched for the term necrotising or necrotizing sarcoid granulomatosis.

    Study Selection: All cases reported in the English literature were included. Read More

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