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    Can granulomatosis with polyangiitis be diagnosed earlier in primary care? A case-control study.
    QJM 2018 Jan;111(1):39-45
    Department of Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK.
    Background: People with granulomatosis with polyangiitis (GPA) commonly described long delays before diagnosis.

    Aim: To study the natural history of GPA prior to diagnosis using primary care data, and determine whether clinical features could be identified to help earlier diagnosis.

    Design: Case-control study using the Clinical Practice Research Datalink. Read More

    Endobronchial Lesion in Eosinophilic Granulomatosis with Polyangiitis.
    Iran J Allergy Asthma Immunol 2017 Dec;16(6):561-564
    Division of Pulmonary and Critical Care, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
    Eosinophilic granulomatosis with polyangiitis is a systemic vasculitis. It could affect respiratory system, kidney, and central nervous system frequently; however, all body organs could be involved. Asthma and eosinophilic pneumonia are predominant manifestations in respiratory system. Read More

    Bronchiectasis is highly prevalent in anti-MPO ANCA-associated vasculitis and is associated with a distinct disease presentation.
    Semin Arthritis Rheum 2017 Dec 7. Epub 2017 Dec 7.
    Service de Médecine Interne, CHU de Nantes, Nantes, France; Centre de Recherche en Transplantation et Immunologie (UMR1064), INSERM, Université de Nantes, Nantes, France.
    Objectives: To assess the prevalence of bronchiectasis in a Western cohort with ANCA-positive granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) and its correlations with disease presentation and outcome.

    Methods: Retrospective study of ANCA-associated vasculitis (AAV) patients followed at Nantes University Hospital (2005-2015). Clinical, biological, and follow-up data were collected through chart review. Read More

    Risk factors and treatment of pneumothorax secondary to granulomatosis with polyangiitis: a clinical analysis of 25 cases.
    J Cardiothorac Surg 2018 Jan 15;13(1). Epub 2018 Jan 15.
    Department of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, 8 Gongren Tiyuchang Nanlu, Chaoyang District, Beijing, 100020, China.
    Objectives: To investigate the risk factors and treatment strategies for pneumothorax secondary to granulomatosis with polyangiitis (GPA).

    Method: Retrospective analysis of cases with pneumothorax secondary to GPA from our own practice and published on literature.

    Results: A total of 25 patients, 18 males and 7 females, mean age 44 ± 15. Read More

    Two clinical cases of granulomatosis with polyangiitis with isolated otitis media and mastoiditis.
    Reumatologia 2017 28;55(5):256-260. Epub 2017 Oct 28.
    MHAT "St. Ivan Rilski", Clinic of Rheumatology, Medical University, Sofia, Bulgaria.
    Granulomatosis with polyangiitis (GPA) is characterised by granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract, often associated with pauci-immune glomerulonephritis. The diagnosis of granulomatosis with polyangiitis is made according to the classification criteria of the ACR criteria for granulomatosis with polyangiitis. We present two cases of granulomatosis with polyangiitis limited/localised form. Read More

    The usefulness of histopathological examinations of non-renal biopsies in the diagnosis of granulomatosis with polyangiitis.
    Reumatologia 2017 28;55(5):230-236. Epub 2017 Oct 28.
    Department and Clinic of Nephrology, Transplantology and Internal Diseases, Medical University of Gdansk, Poland.
    Introduction: Granulomatosis with polyangiitis (GPA) is a rare, ANCA-associated, systemic disease characterized by necrotizing small and medium vessel vasculitis of unknown etiology associated with granulomatous inflammation affecting the renal, pulmonary, upper airways, ocular systems and other tissues. Histological proof of the granulomatosis with polyangiitis (GPA) can be obtained by biopsy of clinically involved sites. The main purpose of this study was to examine histopathological changes in non-renal biopsies from patients with established diagnosis of GPA and evaluated the histological confirmation at diagnosis of this disease. Read More

    Georgian Med News 2017 Dec(273):69-75
    Donetsk National Medical University of Health Ministry of Ukraine, Lyman; State Institution "Dnipropetrovsk Medical Academy of the Ministry of Health of Ukraine", Ukraine.
    The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1. Joint's damage in the form of arthritis or arthralgia observed in 32-67% different groups of patients, that depending on the disease duration, the degree of the pathological process's activity, extraarticular signs severity, lung parenchyma involving and hemodynamic status in the pulmonary circulation. Read More

    Rituximab for anti-neutrophil cytoplasmic antibodies-associated vasculitis: experience of a single center and systematic review of non-randomized studies.
    Rheumatol Int 2018 Jan 10. Epub 2018 Jan 10.
    Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, Cerrahpasa Medical School, Istanbul University, Fatih, Istanbul, Turkey.
    Rituximab (RTX) is becoming a standard treatment for patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) but heterogeneity exists regarding its use. We present our uncontrolled experience with RTX in patients with refractory AAV and also the results of a systematic review of non-randomized studies on RTX in AAV patients. We retrospectively reviewed the records of AAV patients treated with RTX following an inadequate response to immunosuppressives between 2011 and 2015. Read More

    Lung cancer development in the patient with granulomatosis with polyangiitis during long term treatment with cyclophosphamide: first documented case.
    Respirol Case Rep 2018 Feb 22;6(2):e00284. Epub 2017 Dec 22.
    First Department of MedicineTokyo Women's Medical University School of MedicineTokyoJapan.
    A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) at the age of 47, when cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) serology was positive, and he had multiple nodular shadows in both lungs. He had been treated with prednisolone, cyclophosphamide (CPA) and plasma exchange. At the age of 64, a nodular shadow was newly detected in the right lower lung field and serum tumour marker increased. Read More

    Peripheral Ulcerative Keratitis Associated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab.
    Intern Med 2018 Jan 11. Epub 2018 Jan 11.
    Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan.
    A 67-year-old Japanese man was diagnosed with granulomatosis with polyangiitis based on the presence of right maxillary sinusitis, proteinase 3 antineutrophil cytoplasmic antibody positivity, and right scleritis. A conjunctival biopsy specimen showed neutrophil-predominant infiltration around the vessels without granuloma. Because there was a risk of blindness, pulsed methylprednisolone and intravenous cyclophosphamide pulse therapy (IVCY) was started. Read More

    Serum 25-hydroxyvitamin D levels in patients with Granulomatosis with Polyangiitis: association with respiratory infection.
    Clinics (Sao Paulo) 2017 Dec;72(12):723-728
    Divisao de Reumatologia, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR.
    Objectives: To determine the possible association of serum 25-hydroxyvitamin D (25OHD) levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn.

    Methods: Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5) were evaluated in summer/autumn (n=27). The 25OHD levels were measured by radioimmunoassay. Read More

    Clinical and Biological Markers in Hypereosinophilic Syndromes.
    Front Med (Lausanne) 2017 22;4:240. Epub 2017 Dec 22.
    Human Eosinophil Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.
    Hypereosinophilic syndromes (HES) are rare, heterogeneous syndromes characterized by markedly elevated eosinophil counts in the blood and/or tissue and evidence of eosinophil-associated pathology. Although parasitic infections, drug hypersensitivity, and other disorders of defined etiology can present as HES (associated HES), treatment is directed at the underlying cause rather than the eosinophilia itself. A number of additional subtypes of HES have been described, based on clinical and laboratory features. Read More

    Polyps, grommets and eosinophilic granulomatosis with polyangiitis.
    J Laryngol Otol 2018 Jan 9:1-4. Epub 2018 Jan 9.
    Department of Otolaryngology, Head and Neck Surgery,University Hospital Limerick,Ireland.
    Objective: To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.

    Methods: A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis. Read More

    Orbital granulomatosis with polyangiitis masquerading as invasive fungal sinusitis.
    Orbit 2018 Jan 5:1-4. Epub 2018 Jan 5.
    a Stein Eye Institute, University of California , Los Angeles , CA , USA.
    A 55-year-old man presented with unilateral orbital inflammation and no light perception vision. Imaging revealed infiltrative enhancement of the optic nerve, orbit, and intracranial tissue. The case was suspicious for invasive fungal disease, but ultimate workup and orbital biopsy revealed granulomatosis with polyangiitis. Read More

    Colonic perforation due to severe cytomegalovirus disease in granulomatosis with polyangiitis after immunosuppression.
    Clin Rheumatol 2018 Jan 4. Epub 2018 Jan 4.
    Rheumatology Unit, Department of Internal Medicine, Arcispedale Santa Maria Nuova, Azienda USL di Reggio Emilia - IRCCS, Viale Risorgimento 80, 42122, Reggio Emilia, Italy.
    Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Read More

    Granulomatosis with polyangiitis: an unusual cause of acute liver injury.
    BMJ Case Rep 2018 Jan 3;2018. Epub 2018 Jan 3.
    Nephrology Department, BCU Health Board, Wrexham, UK.
    Granulomatosis with polyangiitis (GPA) causing liver injury is rare. Where liver biopsies have been taken findings are not always typical and diagnosis can be challenging. Here, we present a case of a 58-year-old male who on admission to hospital was found to have acute liver injury. Read More

    Appendiceal Perforation in Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss).
    J Clin Rheumatol 2017 Dec 29. Epub 2017 Dec 29.
    Department of Internal Medicine University of Arizona Tucson, AZ Division of Rheumatology Department of Internal Medicine University of Arizona Tucson, AZ Division of Rheumatology Department of Internal Medicine University of Arizona Tucson, AZ.

    Immunoglobulin G4-related Chronic Rhinosinusitis: A Pitfall in the Differential Diagnosis of Granulomatosis with Polyangiitis, Rosai-Dorfman Disease and Fungal Rhinosinusitis.
    Hum Pathol 2017 Dec 26. Epub 2017 Dec 26.
    Department of Otolaryngology Head and Neck Surgery, Beijing TongRen Hospital, Capital Medical University, Beijing, China; Beijing Key Laboratory of Nasal Diseases, Beijing Institute of Otolaryngology, Beijing, China; Department of Allergy, Beijing TongRen Hospital, Capital Medical University, Beijing, China. Electronic address:
    Immunoglobulin G4 (IgG4)-related chronic rhinosinusitis (CRS) has recently been proposed to be a new clinical entity of nasal disease, with no consensually agreed criteria for diagnosis. Moreover, the pathological features if IgG4-related CRS often overlap with other sinonasal inflammatory and autoimmune diseases such as Granulomatosis with Polyangiitis (GPA), Rosai-Dorfman disease (RDD) and Fungal Rhinosinusitis (FRS). We aimed to explore the specific similarities and differences in clinicopathologic features between IgG4-related CRS, and GPA, RDD and FRS, in order that these conditions can be diagnosed more accurately. Read More

    Granulomatosis with Polyangiitis Complicated by Hypertrophic Pachymeningitis Presenting with Simultaneous Multiple Intracerebral Hemorrhages.
    Intern Med 2017 Dec 27. Epub 2017 Dec 27.
    Department of Rheumatology, Tokyo Medical University Hospital, Japan.
    Central nervous system (CNS) involvement in granulomatosis with polyangiitis (GPA), including pachymeningitis and CNS vasculitis, is uncommon. Although intracerebral hemorrhage (ICH) has been reported in GPA, simultaneous multiple ICH (SMICH) is rare. We describe the case of a 50-year-old woman with a history of a limited form of GPA with chronic pachymeningitis who presented with acute-onset headache accompanied by nausea and vomiting, and who developed consciousness impairment. Read More

    Clinical Significance of Neutrophil/lymphocyte Ratio in Patients With Granulomatosis With Polyangiitis.
    Reumatol Clin 2017 Dec 20. Epub 2017 Dec 20.
    Rheumatology Department, Faculty of Medicine, Cairo University, Egypt. Electronic address:
    Objective: To determine to neutrophil-to-lymphocyte ratio (NLR) in granulomatosis with polyangiitis (GPA) patients and to study its relation to disease manifestations and activity.

    Methods: The study included 44 GPA patients and 44 matched age and sex controls. Full history taking, thorough clinical examination with more attention to ocular examination, laboratory and radiological investigations were considered. Read More

    Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Complicated by Perforation of the Small Intestine and Cholecystitis.
    Intern Med 2017 Dec 21. Epub 2017 Dec 21.
    Division of Neurology, Department of Internal Medicine, Tokai University School of Medicine, Japan.
    We report a case of eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) complicated by perforation of the small intestine and necrotizing cholecystitis. A 69-year-old man with a history of bronchial asthma was admitted with mononeuritis multiplex. The laboratory findings included remarkable eosinophilia. Read More

    Eosinophilic granulomatosis with polyangiitis: myocardial thickening reversed by corticosteroids.
    BMC Cardiovasc Disord 2017 Dec 20;17(1):299. Epub 2017 Dec 20.
    Centre for Research and Development, Uppsala University/Region Gävleborg, SE-801 87, Gävle, Sweden.
    Background: In 1951 Churg and Strauss first described the clinical condition now known as eosinophilic granulomatosis with polyangiitis (EGPA), characterized by asthma, nasal polyposis, rhinosinusitis, hypereosinophilia with organ infiltration, and necrotizing vasculitis. It is classified as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, but ANCA negativity is common and more frequently encountered in EGPA with myocardial involvement. Long-term survival has substantially improved with corticosteroid treatment but myocardial involvement is still the leading cause of death in EGPA. Read More

    [Necrotizing sarcoid granulomatosis with clinical presentations of recurrent acute abdomen. Case report and literature review].
    Ter Arkh 2017 ;89(11):60-68
    Research Institute of Eye Diseases, Moscow, Russia.
    The authors have described the world's first case of necrotizing sarcoid granulomatosis (NSG) in a 22-year-old woman with the clinical presentations of acute abdomen, which are associated with abdominal lymph nodal infiltration and necrosis, obvious constitutional disturbances (fever, nocturnal sweats, and significant weight loss), high inflammatory activity (anemia, leukocytosis, high erythrocyte sedimentation rates and C-reactive protein levels), the gradual appearance of splenic and hepatic necrotic foci, and infiltration into the lung and lacrimal glands with the development of unilateral uveitis. The patient underwent five surgical interventions, several needle biopsies for recurrent abdominal syndrome, and long-term antibiotic treatment for presumed sepsis, which had caused drug-induced hepatitis. Bacteriological examination of blood, puncture samples, and removed abdominal cavity tissues, serological tests, and immunomorphogical study of biopsy samples and removed tissues yielded negative results for the presence of bacterial, fungal, and tuberculosis infections. Read More

    Intracerebral hemorrhage after IV tPA for stroke as early symptom of ANCA-associated vasculitis.
    eNeurologicalSci 2017 Dec 1;9:1-2. Epub 2017 Sep 1.
    Department of Neurology, University of Iowa, 200 Hawkins Drive, Iowa City, IA 52242, United States.
    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides. Read More

    (A Critical Appraisal of) Classification of Hypereosinophilic Disorders.
    Front Med (Lausanne) 2017 5;4:216. Epub 2017 Dec 5.
    Université de Lille, INSERM, CHU Lille, U995 - LIRIC - Lille Inflammation Research International Center, Institut d'Immunologie, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Unité d'Immunologie Clinique, Lille, France.
    Hypereosinophilia (HE) is a heterogeneous condition that can be reported in various (namely inflammatory, allergic, infectious, or neoplastic) diseases with distinct pathophysiological pathways. In 1975, Chusid et al. published the first diagnostic criteria of hypereosinophilic syndromes (HES). Read More

    Characteristics and visual outcome of refractory retinal vasculitis associated with antineutrophil cytoplasm antibody-associated vasculitides.
    Am J Ophthalmol 2017 Dec 16. Epub 2017 Dec 16.
    Massachusetts Eye Research and Surgery Institution, Waltham, Massachusetts; Ocular Immunology and Uveitis Foundation, Waltham, Massachusetts; Harvard Medical School, Boston, Massachusetts. Electronic address:
    Purpose: To describe the clinical characteristics, therapies, visual outcomes and prognoses of patients with retinal vasculitis associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV).

    Design: Retrospective case series.

    Methods: Patients diagnosed with retinal vasculitis associated with AAV and at least 6-months of follow-up were included. Read More

    Addison's disease presenting with perimyocarditis.
    J Pediatr Endocrinol Metab 2018 Jan;31(1):101-105
    Department of Pediatric Endocrinology and Diabetes, Great Ormond Street Hospital for Children, UCL Institute of Child Health, London, UK.
    Background: Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.

    Case Presentation: A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.

    Conclusions: The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Read More

    An unusual presentation of eosinophilic angiocentric fibrosis.
    J Surg Case Rep 2017 Dec 8;2017(12):rjx234. Epub 2017 Dec 8.
    Department of Otolaryngology, Head and neck surgery, Royal Prince Alfred Hospital, Camperdown NSW, Australia.
    Eosinophilic angiocentric fibrosis (EAF) is a rare, benign condition affecting the respiratory mucosa and is generally characterized by a locally destructive growth. We present a case of a lady with a saddle nose deformity that had for many years been treated as granulomatosis with polyangiitis (GPA), of which saddle nose deformity is a classic feature. At the time of surgery, she was found to have subglottic stenosis another classic feature of GPA, however, histology demonstrated EAF. Read More

    [What's new in internal medicine?]
    Ann Dermatol Venereol 2017 Dec;144 Suppl 4:IVS23-IVS28
    Service de dermatologie, hôpital Henri-Mondor, 94000 Créteil. Electronic address:
    As it is practiced in France, internal medicine meets the Anglo-Saxon definition of the specialty, ie doctors "equipped to handle the broad and comprehensive spectrum of illnesses that affect adults, and are recognized as experts in diagnosis, in treatment of chronic illness, and in health promotion and disease prevention - they are not limited to one type of medical problem or organ system". This 2017 "What's new in internal medicine" will consist of 2 parts, a first part on significant publications in the field of systemic and autoimmune diseases and a second part on more diverse publications (HIV, cancer, pregnancy, well-being.. Read More

    Pulmonary nodules and nodular scleritis in a teenager with superficial granulomatous pyoderma gangrenosum.
    Pediatr Dermatol 2017 Dec 15. Epub 2017 Dec 15.
    Dermatology Department, National Institute of Pediatrics, Mexico City, Mexico.
    Superficial granulomatous pyoderma gangrenosum, a rare variant of pyoderma gangrenosum, has been considered to be the most benign form of the disease. We present the case of a 15-year-old boy with pulmonary involvement and nodular scleritis associated with this unusual type of pyoderma gangrenosum and discuss its differential diagnosis. Read More

    Anti-Neutrophil Cytoplasmic Antibody-Negative Central Nervous System Granulomatosis With Polyangiitis and Its Clinical Characteristics.
    J Craniofac Surg 2017 Dec 12. Epub 2017 Dec 12.
    Department of Neurosurgery.
    Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions. Read More

    [Granulomatosis with Polyangiitis Complicated with Gastrointestinal Perforation: A Case Report and Review of Literature].
    Nihon Rinsho Meneki Gakkai Kaishi 2017 ;40(5):382-386
    Department of Rheumatology, Yodogawa Christian Hospital.
      A 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes. Read More

    Increased histopathological yield for granulomatosis with polyangiitis based on nasal endoscopy of suspected active lesions.
    Eur Arch Otorhinolaryngol 2017 Dec 11. Epub 2017 Dec 11.
    Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición, Vasco de Quiroga 15, Tlalpan, 14000, Mexico City, Mexico.
    Purpose: To present the results of an endoscopic and histopathologic evaluation of suspected nasal active granulomatosis with polyangiits (GPA) lesions, describe them as seen by the ENT specialist, and propose a guide for tissue sampling of the nasal cavity to improve the yield of confirmatory histology.

    Methods: Randomly selected patients seen from December 1997-October 2007 had a thorough endoscopic nasal evaluation, preceded by careful cleansing of the nasal cavity. Endoscopic lesions were described; sensitivities, specificities, and predictive values of the composites of endoscopic and histological activity were determined. Read More

    Diagnosing alpha-1 antitrypsin deficiency: the first step in precision medicine.
    F1000Res 2017 27;6:2049. Epub 2017 Nov 27.
    Channing Division of Network Medicine, Brigham and Women's Hospital, 181 Longwood Ave., Boston, MA, 02115, USA.
    Severe alpha-1 antitrypsin (AAT) deficiency is one of the most common serious genetic diseases in adults of European descent. Individuals with AAT deficiency have a greatly increased risk for emphysema and liver disease. Other manifestations include bronchiectasis, necrotizing panniculitis and granulomatosis with polyangiitis. Read More

    Granulomatosis With Polyangiitis in a Young Adult With Down Syndrome: Therapeutic Challenges and Clues to Immunopathogenesis.
    J Clin Rheumatol 2017 Dec 1. Epub 2017 Dec 1.
    Section of Rheumatology Yale University School of Medicine New Haven, CT Department of Pathology Yale University School of Medicine New Haven, CT Section of Rheumatology Yale University School of Medicine New Haven, CT

    Granulomatosis With Polyangiitis and Continuous Positive Airway Pressure-The Challenge of Interface Between Nose and Mask.
    J Clin Rheumatol 2017 Dec 1. Epub 2017 Dec 1.
    Departamento de Neurologia e Neurocirurgia, Universidade Federal de São Paulo, São Paulo, Brazil Departamento de Psicobiologia, Universidade Federal de São Paulo, São Paulo, Brazil Departamento de Neurologia e Neurocirurgia, Universidade Federal de São Paulo, São Paulo, Brazil Departamento de Neurologia e Neurocirurgia and Departamento de Psicobiologia, Universidade Federal de São Paulo, São Paulo, Brazil

    Follicular bronchiolitis in an HIV-infected individual on combination antiretroviral therapy with low CD4+ cell count but sustained viral suppression.
    BMJ Case Rep 2017 Nov 29;2017. Epub 2017 Nov 29.
    Department of Respiratory Medicine, Odense University Hospital, Odense, Denmark.
    A 36-year-old Danish man, living in Asia, was diagnosed with Pneumocystis pneumonia (PCP) and HIV in 2013 (CD4+ count: 6 cells/µL; viral load: 518 000 copies/mL). He initiated combination antiretroviral therapy. Later that year, he was also diagnosed with granulomatosis with polyangiitis and was treated with prednisolone. Read More

    Eosinophilic granulomatosis with polyangiitis without respiratory symptoms or asthma in an adolescent: case report and literature review.
    Rheumatol Int 2017 Nov 30. Epub 2017 Nov 30.
    Department of Pediatric Rheumatology, Pamukkale University School of Medicine, Pamukkale, Denizli, Turkey.
    Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. Read More

    Renal outcome of kidney-transplantation in Korean recipients with ANCA-associated vasculitis.
    Clin Exp Rheumatol 2017 Nov 27. Epub 2017 Nov 27.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
    Objectives: We investigated renal outcome of kidney-transplantation in Korean recipients with biopsy-proven renal involvement of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a single centre.

    Methods: We reviewed the medical records of 144 Korean patients and included 3 female patients with microscopic polyangiitis (MPA) and one male patient with eosinophilic granulomatosis with polyangiitis (EGPA) in this study. We obtained clinical and laboratory data related to kidney-transplantation, analysed renal outcome of kidney-transplantation in 4 recipients with AAV and compared it with those of previous studies. Read More

    The utility of the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis in Korean patients with antineutrophil cytoplasmic antibody-associated vasculitis.
    Clin Exp Rheumatol 2017 Nov 27. Epub 2017 Nov 27.
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
    Objectives: We applied the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis (GPA) to 150 Korean patients with previously diagnosed antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and investigated how many patients with AAV were reclassified as GPA.

    Methods: We included patients with 30 GPA, 30 eosinophilic GPA (EGPA) and 90 microscopic polyangiitis (MPA) patients. Patients can be classified as GPA, when the sum of scores is more than 5. Read More

    Childhood- versus adult-onset ANCA-associated vasculitides: A nested, matched case-control study from the French Vasculitis Study Group Registry.
    Autoimmun Rev 2017 Nov 24. Epub 2017 Nov 24.
    National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (APHP), Paris, France; Université Paris Descartes, Paris, France. Electronic address:
    Objective: To investigate differences between childhood-onset ANCA-associated vasculitides (cAAVs) and matched adult-onset controls (aAAVs).

    Methods: cAAV clinical pictures at onset and outcomes were compared to a randomly selected sample of aAAV patients from the French Vasculitis Study Group Registry. Cases and controls were matched for AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] or eosinophilic granulomatosis with polyangiitis [EGPA]), sex and year of enrollment. Read More

    Eosinophilic Myocarditis.
    Am J Med Sci 2017 Nov 6;354(5):486-492. Epub 2017 Apr 6.
    Division of Hematology, University of British Columbia, Vancouver, British Columbia. Electronic address:
    Persistent eosinophilia can cause cardiac tissue damage, typically in the form of eosinophilic myocarditis, whether the underlying cause is reactive, a clonal myeloid disorder, or idiopathic hypereosinophilic syndrome (HES). Eosinophilic myocarditis ranges from mild localized disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications and endomyocardial fibrosis. Systemic treatment varies widely depending on the underlying cause, so thorough investigation and precise diagnosis are essential. Read More

    Eosinophilic myocarditis as a first presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
    BMJ Case Rep 2017 Nov 23;2017. Epub 2017 Nov 23.
    Department of Cardiology, Mercy University Hospital, Cork, Ireland.
    We present the case of a 28-year-old man who presented with chest pain and elevated cardiac biomarkers, with no evidence of acute ischaemia. He had a pronounced eosinophilia, abnormal echocardiographic, cardiac MRI and CT findings. He underwent transbronchial biopsy of carinal lymph nodes and of lung parenchyma. Read More

    Association of ETS1 polymorphism with granulomatosis with polyangiitis and proteinase 3-anti-neutrophil cytoplasmic antibody positive vasculitis in a Japanese population.
    J Hum Genet 2018 Jan 5;63(1):55-62. Epub 2017 Oct 5.
    Molecular and Genetic Epidemiology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.
    ETS proto-oncogene 1, transcription factor (ETS1) is involved in various immune responses. Genome-wide association studies on systemic lupus erythematosus in Chinese populations identified the association of ETS1 polymorphism in 3' untranslated region, rs1128334A, which was associated with lower ETS1 expression. In view of substantial sharing of susceptibility genes across multiple autoimmune diseases, we examined whether ETS1 is associated with a rare autoimmune rheumatic disease, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Read More

    Gastrointestinal and Hepatic Disease in Vasculitis.
    Rheum Dis Clin North Am 2018 Feb;44(1):1-14
    Denver Veterans Affairs Medical Center, University of Colorado School of Medicine, Denver, CO, USA. Electronic address:
    Vasculitis is an inflammatory condition that targets the blood vessels, which may occur in isolation or as a component of a systemic inflammatory condition. Although many of the vasculitides can directly affect the organs of the gastrointestinal system, some types exhibit a proclivity for certain gastrointestinal and hepatic organs. Often a patient presents with nonspecific symptoms, delaying the diagnosis and treatment of the underlying vasculitis. Read More

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

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