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    Imatinib mesylate use in refractory eosinophilic granulomatosis with polyangiitis: a literature review and a case report.
    Clin Rheumatol 2018 Mar 21. Epub 2018 Mar 21.
    Department of Vascular Problems in Rheumatic Diseases, V.A. Nasonova Research Institute of Rheumatology, Kashirskoe shosse 34A, Moscow, Russian Federation, 115522.
    Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Read More

    A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs.
    Case Rep Med 2018 16;2018:3124281. Epub 2018 Jan 16.
    University Medical Unit, Colombo South Teaching Hospital, Kalubowila, Sri Lanka.
    Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. Read More

    [Musculoskeletal symptoms in a group of granulomatosis with polyangiitis patients].
    Wiad Lek 2018 ;71(1 pt 1):17-20
    Klinika Reumatologii i Układowych Chorób Tkanki Łącznej, Uniwersytet Medyczny w Lublinie, Lublin, Polska.
    Objective: Introduction: Musculoskeletal symptoms are not characteristic for granulomatosis with polyangiitis (GPA) patients. However, they could be present in two-thirds of patients at the onset of the disease and may provoke diagnostic difficulties. The aim: To assess retrospectively musculoskeletal manifestations in a group of GPA patients. Read More

    Birmingham vasculitis activity and chest manifestation at diagnosis can predict hospitalised infection in ANCA-associated vasculitis.
    Clin Rheumatol 2018 Mar 20. Epub 2018 Mar 20.
    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 development rate and time, risk factors, predictors, and aetiologies of hospitalised infection in Korean patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We retrospectively reviewed the medical records of 154 patients with AAV. Hospitalised infection was considered only when patients were admitted for serious infection related to AAV or AAV treatment. Read More

    Rathke's cleft cyst associated with pituitary granulomatosis with polyangiitis: An unusual combination of hypothalamus-pituitary region pathologies.
    Radiol Case Rep 2018 Feb 31;13(1):233-236. Epub 2017 Oct 31.
    Department of Neurosurgery, Fondazione IRCCS "Ca' Granda" Ospedale Maggiore Policlinico, Milan, Italy.
    The authors present an unusual case of a patient suffering from visual deficit due to pituitary granulomatosis with polyangiitis (GPA) associated with Rathke's cleft cyst (RCC). The patient was referred to our Neurosurgery Department presenting right eye amaurosis, third cranial nerve palsy, and left temporal hemianopsia. Magnetic resonance imaging documented a sellar or suprasellar lesion with solid and cystic components. Read More

    Nasal polyposis pathophysiology: Endotype and phenotype open issues.
    Am J Otolaryngol 2018 Mar 7. Epub 2018 Mar 7.
    Department of Neuroscience DNS, Otolaryngology Section, Padova University, Padova, Italy.
    Purpose: Endotyping chronic rhinosinusitis with nasal polyps (CRSwNP) poses a challenge for rhinologists nowadays. Phenotyping CRSwNP proved inappropriate as an approach to their classification because of their common clinical features. Endotyping, being based on the pathogenic mechanism, provides a precise picture more appropriate for use in clinical practice. Read More

    Granulomatosis with Polyangiitis Involving the Ascending Aorta.
    Ann Thorac Surg 2018 Mar 9. Epub 2018 Mar 9.
    Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea. Electronic address:
    A 58-year-old man visited the emergency department with vague anterior chest discomfort. Based on computed tomographic findings, our initial suspicion was intramural hematoma (IMH) in the ascending aorta. After a comprehensive discussion with relevant departments, emergent operation was planned. Read More

    Reducing glucocorticoid duration in ANCA-associated vasculitis: A pilot trial.
    Semin Arthritis Rheum 2018 Feb 3. Epub 2018 Feb 3.
    Massachusetts General Hospital, Department of Medicine, Division of Rheumatology, Yawkey Center for Outpatient Care, Suite 2C, Boston, MA 02114.
    Objective: Therapeutic advances in ANCA-associated vasculitis (AAV) have improved patient survival, but mortality rates remain higher than the general population. Glucocorticoids contribute to AAV morbidity and mortality. We examined whether an 8-week glucocorticoid course in combination with rituximab (RTX) would induce disease remission in patients with AAV. Read More

    ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery.
    Case Rep Otolaryngol 2017 30;2017:9687383. Epub 2017 Nov 30.
    Department of Biology and Genetics, Laboratory of Cancer Medical Science, Hokuto Hospital, Inadacho Kisen 7-5, Obihiro 080-0833, Japan.
    We report a rare case of granulomatosis with polyangiitis (GPA) presenting with hypertrophic cranial pachymeningitis (HCP), abducens nerve palsy, and stenosis of the internal carotid artery (ICA). A 59-year-old Japanese man presented with a year history of nasal obstruction and a 2-month history of slight headache. Histopathological examination of the granulomatous mucosa in the ethmoid sinuses resected by endoscopic sinus surgery revealed necrotizing vasculitis with multinucleated giant cells. Read More

    Novel insights into the aetiology of granulomatosis with polyangiitis-a case-control study using the Clinical Practice Research Datalink.
    Rheumatology (Oxford) 2018 Feb 26. Epub 2018 Feb 26.
    Division of Epidemiology and Public Health, University of Nottingham, UK.
    Objectives: We aimed to provide insights into the aetiology of granulomatosis with polyangiitis (GPA), by conducting a large case-control study using a general population-based, prospectively collected database of healthcare records.

    Methods: We compared all incident cases of GPA in the Clinical Practice Research Datalink 1990-2014, with up to 10 age-, sex- and general practice-matched controls. We identified potential risk factors, recorded numbers of cases and controls exposed to each, and calculated odds ratios (ORs) using conditional logistic regression. Read More

    Temporary Abdominal Closure Combined With an Irrigating System Utilizing Hypochlorous Acid Solution to Decrease Abdominal Mucopurulence.
    Eplasty 2018 26;18:e12. Epub 2018 Feb 26.
    Department of Surgery, Maricopa Integrated Health System, Phoenix, AZ.
    Leaving the abdominal cavity open is a well-described and frequently utilized technique in the treatment of severe intra-abdominal sepsis. Irrigation through a negative pressure wound therapy device is a technique employed to assist in the closure of wounds as well as the reduction of bacterial contamination. Furthermore, hypochlorous acid has been found to be safe and effective in microorganismal elimination from extremity wounds. Read More

    Rituximab-induced Acute Thrombocytopenia in Granulomatosis with Polyangiitis.
    Intern Med 2018 Mar 9. Epub 2018 Mar 9.
    Department of Rheumatology, Unit of Advanced Preventive Medical Sciences, Graduate School of Biomedical Sciences, Nagasaki University, Japan.
    A 72-year-old Japanese woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis was admitted to our hospital with hearing loss, temporal pain, and sudden blindness. We finally diagnosed recurrent granulomatosis with polyangiitis and initiated methyl-prednisolone pulse therapy (1,000 mg) followed by prednisolone (30 mg/day) and rituximab (RTX). After the third RTX administration, she developed bloody stools along with acute thrombocytopenia and low complement levels. Read More

    Anti-neutrophil cytoplasmic antibodies and their clinical significance.
    Clin Rheumatol 2018 Mar 10. Epub 2018 Mar 10.
    Center of Excellence in Immunology and Immune-mediated diseases, Department of Microbiology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
    Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay. Read More

    Determinants of renal and patient outcomes in a Spanish cohort of patients with ANCA-associated vasculitis and renal involvement.
    Clin Rheumatol 2018 Mar 9. Epub 2018 Mar 9.
    Nephrology Division, Hospital Universitari Fundació Puigvert, Universitat Autònoma de Barcelona, Barcelona, Spain.
    The classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains controversial. The main objective of this study was to define the respective values of ANCA serotype-based classification, clinicopathological classification, and histopathological classification in predicting patient and renal outcomes in a Spanish cohort of patients with ANCA with specificity for myeloperoxidase, MPO-ANCA, versus ANCA with specificity for proteinase 3, PR3-ANCA. Two hundred and forty-five patients with ANCA-AAV and biopsy-proven renal involvement diagnosed between 2000 and 2104 were recruited in 12 nephrology services. Read More

    Cavitary Lung Diseases-A Clinical-Radiological Algorithmic Approach.
    Chest 2018 Mar 5. Epub 2018 Mar 5.
    Cavities are occasionally encountered on thoracic imaging. Their differential diagnosis is large and includes, among others, various infections, autoimmune conditions and primary and metastatic malignancies. We offer an algorithmic approach to their evaluation by initially excluding mimics of cavities then broadly classifying them according to the duration of clinical symptoms and radiological abnormalities. Read More

    Diagnostic and clinical role of serum proteinase 3 antineutrophil cytoplasmic antibodies in inflammatory bowel disease.
    J Gastroenterol Hepatol 2018 Mar 7. Epub 2018 Mar 7.
    Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi. Kurume, Fukuoka, 830-0011, Japan.
    Background And Aim: Proteinase 3 antineutrophil cytoplasmic antibodies (PR3-ANCAs) are well-known serological markers for granulomatosis with polyangiitis, but their role as serological markers for inflammatory bowel disease (IBD) remains uncertain. The present study aimed to evaluate the diagnostic and clinical role of PR3-ANCAs as markers for IBD.

    Methods: Using a new methodology with chemiluminescence enzyme immunoassay, serum PR3-ANCA titres were assessed in 102 patients with ulcerative colitis (UC), 67 patients with Crohn's disease (CD), 44 controls with other intestinal diseases and 66 healthy controls. Read More

    Progressive multifocal leukoencephalopathy in rituximab-treated rheumatic diseases: a rare event.
    J Neurovirol 2018 Mar 5. Epub 2018 Mar 5.
    Roche Products Ltd., Welwyn Garden City, UK.
    This report assesses the observed risk of PML in patients treated with the anti-CD20 monoclonal antibody rituximab in the regulatory authority-approved autoimmune indications rheumatoid arthritis (RA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA). This was a cumulative analysis of confirmed PML cases in patients receiving rituximab for RA or GPA/MPA from both spontaneous reports and clinical trial sources, as captured in the manufacturer global company safety and clinical databases. Overall reporting rates were calculated and patient case details were summarized. Read More

    Granulomatosis with polyangiitis presenting as facial nerve palsy in a teenager.
    Int J Pediatr Otorhinolaryngol 2018 Apr 7;107:160-163. Epub 2018 Feb 7.
    Department of Otolaryngology - Head and Neck Surgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA; Division of Pediatric Otolaryngology - Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Electronic address:
    Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e. Read More

    [Scleritis and systemic diseases: What should know the internist?]
    Rev Med Interne 2018 Feb 26. Epub 2018 Feb 26.
    Service de médecine interne, hospices civils de Lyon, hôpital de la Croix-Rousse, 103, grande rue de la Croix-Rousse, 69317 Lyon cedex 04, France; Université de Lyon, 69100 Lyon, France. Electronic address:
    Scleritis is an inflammatory disease of the sclera; outer tunic of the eye on which the oculomotor muscles are inserted. It can be associated with a systemic disease up to one time out of 3. These associated diseases are mainly rheumatoid arthritis, vasculitis, including granulomatosis with polyangiitis in the first line and spondyloarthropathies. Read More

    A case of squamous cell carcinoma of the nasal cavity in a patient with granulomatosis with polyangiitis (Wegener granulomatosis).
    Ear Nose Throat J 2018 Jan-Feb;97(1-2):E37-E41
    Department of Head and Neck Surgery, Ronald Reagan UCLA Medical Center, 10833 Le Conte Ave., 62-132 CHS, Los Angeles, CA 90095, USA.
    We report a rare case of squamous cell carcinoma (SCC) of the nasal cavity arising in a patient with granulomatosis with polyangiitis (GPA). The patient was a 35-year-old man who had been diagnosed 15 years earlier with GPA and treated medically for sinonasal, pulmonary, and renal involvement. He presented to us with left-sided orbital and cheek pain and nasal obstruction. Read More

    Granulomatosis with polyangiitis: clinical course and outcome of 60 patients from a single center in South India.
    Clin Exp Med 2018 Feb 28. Epub 2018 Feb 28.
    Department of Nephrology, St. John's National Academy of Health Sciences, Bengaluru, India.
    Granulomatosis with polyangiitis (GPA) previously known as Wegener's granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Read More

    [The modern approach to the treatment of subglottic laryngeal stenosis].
    Vestn Otorinolaringol 2018 ;83(1):52-55
    L.I. Sverzhevskiy Research Institute of Clinical Otorhinolaryngology, Moscow Health Department, Moscow, Russia, 117152.
    The objective of the present study was to optimize the treatment of patients with subglottic laryngeal stenosis.

    Material And Methods: We have examined a total of 14 patients presenting with subglottic laryngeal stenosis. The etiological factors and underlying conditions of laryngeal stenosis were the prolonged intubation in 4 patients, Wegener granulomatosis in 6 patients, and idiopathic stenosis of the larynx in 4 patients. Read More

    Development of Hypertrophic Pachymeningitis in a Patient With Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis.
    J Clin Rheumatol 2018 Feb 27. Epub 2018 Feb 27.
    From the Department of Nephrology, Rheumatology, Endocrinology, and Metabolism, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.

    Costs of Disease Relapses Among Individuals with Granulomatosis, with Polyangiitis, or Microscopic Polyangiitis in the United States.
    Rheumatol Ther 2018 Feb 22. Epub 2018 Feb 22.
    Genentech, Inc, South San Francisco, CA, USA.
    Introduction: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are two related forms of systemic vasculitis. Patients with these conditions often experience relapses affecting various body systems. Here we describe rates of relapse and review healthcare costs resulting from relapse among patients with GPA/MPA. Read More

    A case of probable trimethoprim-sulfamethoxazole induced circulating antineutrophil cytoplasmic antibody-positive small vessel vasculitis.
    Dermatol Online J 2017 Aug 15;23(8). Epub 2017 Aug 15.
    Department of Dermatology, University of Illinois College of Medicine at Peoria, Peoria, Illinois.
    Cutaneous leukocytoclastic vasculitis (LCV) can occur as skin-limited disease or as part a systemic vasculitis. Appropriate workup includes the evaluation of antineutrophil cytoplasmic antibodies (ANCAs), with a positive titer raising concern for the associated primary vasculitides including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), or eosinophilic granulomatosis with polyangiitis (EGPA). In the absence of systemic findings, however, a drug etiology must also be considered. Read More

    The initial predictors of death in 153 patients with ANCA-associated vasculitis in a single Korean centre.
    Clin Exp Rheumatol 2018 02 14. Epub 2018 Feb 14.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
    Objectives: We estimated the cumulative patient survival rates, the causes of death and the initial predictors of death in Korean patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA).

    Methods: We reviewed the medical records of 153 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We collected clinical and laboratory data including ANCA, Birmingham vasculitis activity score (BVAS), five factor score (FFS) (2009), comorbidities, medications and prognosis (death and relapse). Read More

    Cancer development in Korean patients with ANCA-associated vasculitis: a single centre study.
    Clin Exp Rheumatol 2018 Feb 14. Epub 2018 Feb 14.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul; and Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Republic of Korea.
    Objectives: We investigated the incidence rate and type of cancer, and furthermore, estimated the standardised incidence ratios (SIRs) of cancer in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Korea.

    Methods: We retrospectively included 150 patients with AAV [81 patients with microscopic polyangiitis (MPA), 38 with granulomatosis with polyangiitis (GPA) and 31 patients with eosinophilic GPA (EGPA)], and reviewed their medical records. We collected demographic, clinical and laboratory data, and reviewed the use of glucocorticoid and immunosuppressive drugs administered until detection of cancer or last visit. Read More

    SERPINA1 Gene Variants in Granulomatosis with Polyangiitis.
    Adv Exp Med Biol 2018 Feb 20. Epub 2018 Feb 20.
    Department of Family Medicine, Internal and Metabolic Diseases, Warsaw Medical University, Warsaw, Poland.
    Alpha-1 antitrypsin (A1AT) deficiency is one of the most common genetic disorders in Caucasian population. There is a link between granulomatosis with polyangiitis (GPA) and most frequent variants of SERPINA1 gene encoding severe alpha-1 antitripsin deficiency. However, the potential effect of Pi*Z, Pi*S as well as other SERPINA1 variants on clinical course of vasculitis are not well understood. Read More

    Ear, nose and throat involvement in granulomatosis with polyangiitis: how it presents and how it determines disease severity and long-term outcomes.
    Clin Rheumatol 2018 Feb 20. Epub 2018 Feb 20.
    Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Via Giustiniani, 2, 35128, Padova, Italy.
    Ear, nose and throat (ENT) manifestations in granulomatosis with polyangiitis (GPA) represent the most frequent symptoms at disease onset. The aim of the study was to analyse ENT involvement at diagnosis, as well as how it could influence relapse rate, mortality and disease severity. A retrospective non-controlled cohort study was performed including all consecutive diagnosed GPA from 1996 to 2016 in two rheumatology centres of Northern Italy, focusing particularly on ENT presenting signs and symptoms at baseline. Read More

    A case of renal granulomatosis with polyangiitis following intravesical bacillus Calmette-Guérin therapy.
    Saudi J Kidney Dis Transpl 2018 Jan-Feb;29(1):185-188
    Department of Nephrology, La Rabta Hospital, Jebbari, Tunis, Tunisia.
    Various adverse reactions may occur after intravesical bacillus Calmette-Guérin (BCG) therapy. Although the virulence of attenuated BCG is low, serious complications such as bacterial cystitis, bladder contractures, granulomatous prostatitis, epididymitis, orchitis, and systemic reactions such as fever and malaise have been described. Disseminated granulomatosis such as hepatitis and pneumonitis have also been described, but are rare. Read More

    Breast granulomatosis with polyangiitis mimicking breast cancer.
    Pathologica 2017 Dec;109(4):405-407
    Pathology Unit, Azienda USL Valle d'Aosta, Hospital "Parini", Aosta, Italy.
    Inflammatory lesions of the breast encompass primary reactive processes and local manifestation of systemic diseases. They are very rare and they are generally treated without resort to biopsy. Nevertheless they could be clinically challenge mimicking malignant process and needing surgery to reach a correct diagnosis. Read More

    Linked help from bacterial proteins drives autoantibody production in small vessel vasculitis.
    Med Hypotheses 2018 Mar 17;112:24-26. Epub 2018 Jan 17.
    St George's, University of London, Cranmer Terrace, Tooting, London SW17 0RE, United Kingdom. Electronic address:
    The small vessel vasculitides granulomatosis with polyangiitis (GPA) and microscopic polyangiitis are associated with autoantibodies to neutrophil cytoplasm antigens (ANCA), principally proteinase-3 (PR3) and myeloperoxidase (MPO). There is an association between GPA and nasal carriage of Staphylococcus aureus. The recent finding that S. Read More

    Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis. Case Report and Review of the Recent Literature.
    Headache 2018 Feb 15. Epub 2018 Feb 15.
    1st Neurology Department, Aristotle University, AHEPA Hospital, Thessaloniki, Greece.
    Objective: The objective of this article is to report a rare case of headache as the initial symptom of granulomatosis with polyangiitis (GPA) and to review the recent literature.

    Background: Granulomatosis with polyangiitis is a rare, systemic, autoimmune disease of unknown etiology. GPA has a wide spectrum of clinical symptomatology, including involvement of the nervous system, even as the initial manifestation. Read More

    Red Blood Cell Distribution Width Can Predict Vasculitis Activity and Poor Prognosis in Granulomatosis with Polyangiitis.
    Yonsei Med J 2018 Mar;59(2):294-302
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
    Purpose: We investigated whether red blood cell distribution width (RDW) predicts vasculitis activity based on Birmingham vasculitis activity score (BVAS) or BVAS for granulomatosis with polyangiitis (GPA) at diagnosis and poor prognosis during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

    Materials And Methods: We reviewed the medical records of 150 patients with AAV. We defined severe GPA as BVAS for GPA ≥7 (the highest quartile). Read More

    A Case of Suspected Hepatically Localized Granulomatosis with Polyangiitis.
    Intern Med 2018 Feb 9. Epub 2018 Feb 9.
    Department of Anatomic Pathology, Kurashiki Central Hospital, Japan.
    The patient was a 72-year-old woman whose alkaline phosphatase levels had been elevated since she was 56 years old. Liver biopsies obtained when the patient was 64 and 66 years of age led to a suspicion of cholangitis caused by vasculitis. Furthermore, proteinase-3 anti-neutrophil cytoplasmic antibody positivity led to a suspicion of granulomatosis with polyangiitis, but subjective symptoms and disorders in other organs were absent, so this suspicion was not confirmed. Read More

    Comment on the article entitled "Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome" (Autoimmunity Reviews 16 (2017) 1036-1043).
    Autoimmun Rev 2018 Feb 8. Epub 2018 Feb 8.
    St Eloi Hospital, Department of Internal Medicine and Multi-organic Diseases, Local Referral Center for Systemic and Autoimmune Diseases, 80 Avenue Augustin Fliche, 34295 Montpellier, France; Montpellier University, Medical School, Montpellier F-34967, France; INSERM U1183, St Eloi Hospital, 80 Avenue Augustin Fliche, 34295 Montpellier, France. Electronic address:

    Frequency, Diagnosis, Treatment, and Outcome of Gastrointestinal Disease in Granulomatosis with Polyangiitis and Microscopic Polyangiitis.
    J Rheumatol 2018 Feb 1. Epub 2018 Feb 1.
    From the Department of Rheumatology, and the Department of Surgery, Department of Clinical and Experimental Medicine, and the Department of Nephrology, Department of Medical and Health Sciences, Linköping University; Department of Rheumatology, and Department of Nephrology, County Council of Östergötland, Linköping, Sweden. P. Eriksson, associate professor, Department of Rheumatology, Linköping University hospital; M. Segelmark, professor, Department of Nephrology, Linköping University hospital; O. Hallböök, professor, Department of Surgery, Linköping University hospital. Address correspondence to P. Eriksson, Dept. of Rheumatology, Linköping University hospital, 581 85 Linköping, Sweden. E-mail: Accepted for publication October 24, 2017.
    Objective: Involvement of the gastrointestinal (GI) tract is a rare complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The aim was to describe frequency, diagnosis, treatment, and outcome of GI disease in a large series of patients in a single center.

    Methods: A database that includes all patients with GPA and MPA diagnosed since 1997 in a defined area of southeastern Sweden as well as prevalent older cases and tertiary referral patients was screened for patients with GI disease. Read More

    Epidemiology of primary systemic vasculitis in children: a population-based study from southern Sweden.
    Scand J Rheumatol 2018 Feb 7:1-8. Epub 2018 Feb 7.
    d Department of Rheumatology, Clinical Sciences Lund , Lund University , Lund , Sweden.
    Objectives: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden.

    Methods: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1. Read More

    [Management of orbital inflammation in internal medicine. Proposal for a diagnostic work-up].
    Rev Med Interne 2018 Feb 1. Epub 2018 Feb 1.
    Service de médecine interne, hôpital de la Croix-Rousse, hospices Civils de Lyon, université de Lyon, 103, grande rue de la Croix-Rousse, 69317 Lyon cedex 04, France. Electronic address:
    Inflammatory orbitopathies relate to an inflammatory state originating within the orbit and its adnexes, except the inner ocular globe. Orbital inflammation (OI) may be either localized manifestation of a proven or like autoimmune disease, or local response from immune system against infectious, structural or tumoral antigens. We review the clinical manifestations of OI, which provide helpful clues to the diagnosis and describe the inflammatory, infectious and neoplastic conditions classically associated with OI. Read More

    Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature.
    Int Arch Allergy Immunol 2018 23;175(1-2):61-69. Epub 2018 Jan 23.
    Respiratory Pathophysiology Unit, Cardiothoracic and Vascular Department, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.
    Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. Read More

    When a wound is the harbinger of a serious underlying systemic illness.
    BMJ Case Rep 2018 Jan 31;2018. Epub 2018 Jan 31.
    Department of Rheumatology, University of Mississippi Medical Center, Jackson, Mississippi, USA.
    A 55-year-old woman presented with progressive enlarging and painful non-healing ulcers on her bilateral lower extremities; biopsy was consistent with pyoderma gangrenosum. Workup for an underlying illness revealed a cavitary lung nodule and an ulcerating mass in the anal canal. Patient did not have any respiratory or gastrointestinal symptoms. Read More

    Otologic Manifestations and Progression in Patients with Wegener's granulomatosis: A Survey in 55 Patients.
    Iran J Otorhinolaryngol 2017 Nov;29(95):327-331
    Orthopedic Surgeon, Akhtar Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    Introduction: Granulomatosis with polyangiitis (GPA; also known as Wegener's granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otology manifestations in the disease course.

    Materials And Methods: In the current prospective study, patients with a diagnosis of GPA from 2012-2016 were included. Read More

    Health-related quality of life in ANCA-associated vasculitis and item generation for a disease-specific patient-reported outcome measure.
    Patient Relat Outcome Meas 2018 4;9:17-34. Epub 2018 Jan 4.
    Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
    Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are multisystem diseases of the small blood vessels. Patients experience irreversible damage and psychological effects from AAV and its treatment. An international collaboration was created to investigate the impact of AAV on health-related quality of life (HRQoL), and develop a disease-specific patient-reported outcome measure to assess outcomes of importance to patients. Read More

    Multiple Cerebral Infarctions Due to Patent Foramen Ovale in a Patient with Eosinophilic Granulomatosis with Polyangiitis.
    J Stroke Cerebrovasc Dis 2018 Jan 17. Epub 2018 Jan 17.
    Department of Neurosurgery, Ise Red Cross Hospital, Ise-city, Mie, Japan.
    A 51-year-old man was diagnosed with eosinophilic granulomatosis with polyangiitis 6 years ago due to asthma, sinusitis, hypereosinophilia, and peripheral neuropathy based on the diagnostic criteria of American College of Rheumatology, and corticosteroid therapy achieved a remission. One year ago, he was hospitalized due to deep venous thrombosis (DVT) and pulmonary embolism, and rivaroxaban was administrated. He was admitted to our hospital for acute onset of diplopia and right hemiparesis. Read More

    The Utility of Urinalysis in Determining the Risk of Renal Relapse in ANCA-Associated Vasculitis.
    Clin J Am Soc Nephrol 2018 Feb 25;13(2):251-257. Epub 2018 Jan 25.
    Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.
    Background And Objectives: The significance of persistent hematuria or proteinuria in patients with ANCA-associated vasculitis who are otherwise in clinical remission is unclear.

    Design, Setting, Participants, & Measurements: Aanalysis was conducted using participants enrolled in two randomized, placebo-controlled clinical trials who had active GN due to ANCA-associated vasculitis, had positive ANCA, and achieved remission by month 6. Dipstick and microscopic urinalyses were performed at each visit. Read More

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