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    Successful reconstruction of an ocular defect resulting from granulomatosis with polyangiitis, following treatment with rituximab.
    Am J Ophthalmol Case Rep 2018 Jun 15;10:240-243. Epub 2018 Mar 15.
    Mater Misericordiae University Hospital, Eccles St, Dublin 7, Ireland.
    Purpose: To report a unique case of orbital inflammatory disease which was ultimately diagnosed as granulomatosis with polyangitis (GPA) and thus successfully treated.

    Observation: A 47 year-old man presented with a rapidly progressive necrotic soft tissue mass within the medial antero-superior aspect of the right eyelid and orbit. He also had transient retinal vasculitis in the left. Read More

    Mycophenolate mofetil for induction and maintenance of remission in naïve patients with granulomatosis with polyangiitis without renal involvement.
    Reumatol Clin 2018 May 16. Epub 2018 May 16.
    Faculty of Behavioral, Management and Social sciences, Department Psychology, Health and Technology, University of Twente, Enschede, The Netherlands.
    Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up. Read More

    Granulomatosis with polyangiitis: a 17 year experience from a tertiary care hospital in Pakistan.
    BMC Res Notes 2018 May 16;11(1):303. Epub 2018 May 16.
    Department of Medicine, Aga Khan University Hospital, Stadium Road, Karachi, 74800, Pakistan.
    Objective: Granulomatosis with Polyangiitis (GPA) is an autoimmune, multi-system, small and medium vessel vasculitis with granulomatous inflammation. Aim of this study was to assess the clinical and radiological presentations of patients with GPA amongst the Pakistani population. It is a single centre retrospective single observation study. Read More

    Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review.
    Drugs 2018 May 15. Epub 2018 May 15.
    Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Université Paris Descartes, Paris, France.
    Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis. Nevertheless, eosinophilic granulomatosis with polyangiitis stands apart because it has features of vasculitis and eosinophilic disorders that require targeted therapies somewhat different from those used for other anti-neutrophil cytoplasm antibody-associated vasculitides. Considerable advances have been made in understanding the underlying pathophysiology of eosinophilic granulomatosis with polyangiitis that have highlighted the key role of eosinophils and opened new therapeutic opportunities. Read More

    Improved survival with renal transplantation for end-stage renal disease due to granulomatosis with polyangiitis: data from the United States Renal Data System.
    Ann Rheum Dis 2018 May 14. Epub 2018 May 14.
    Rheumatology Unit, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.
    Background: Renal transplantation is the optimal treatment for selected patients with end-stage renal disease (ESRD). However, the survival benefit of renal transplantation among patients with ESRD attributed to granulomatosis with polyangiitis (GPA) is unknown.

    Methods: We identified patients from the United States Renal Data System with ESRD due to GPA (ESRD-GPA) between 1995 and 2014. Read More

    Proteinase 3 Interferes With C1q-Mediated Clearance of Apoptotic Cells.
    Front Immunol 2018 25;9:818. Epub 2018 Apr 25.
    INSERM U1016, Cochin Institute, Paris, France.
    Proteinase 3 (PR3) is the autoantigen in granulomatosis with polyangiitis, an autoimmune necrotizing vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Moreover, PR3 is a serine protease whose membrane expression can potentiate inflammatory diseases such as ANCA-associated vasculitis and rheumatoid arthritis. During apoptosis, PR3 is co-externalized with phosphatidylserine (PS) and is known to modulate the clearance of apoptotic cells through a calreticulin (CRT)-dependent mechanism. Read More

    HBsAg-negative and anti-HBc-positive in eosinophilic granulomatosis with polyangiitis: a retrospective pilot study.
    Rheumatol Int 2018 May 12. Epub 2018 May 12.
    Division of Gastroenterology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea.
    We examined whether resolved hepatitis B virus (HBV) infection was associated with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), and affected AAV activity at diagnosis and prognosis during the follow-up. We reviewed the electronic medical records of 153 AAV patients, and included 91 hepatitis B surface antigen (HBsAg)-negative patients having results of both antibody to hepatitis B core antigen (anti-HBc) and surface antigen (anti-HBs). We collected clinical and laboratory data, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis and relapse rates during the follow-up. Read More

    The presence of staphylococcal superantigens in nasal swabs and correlation with activity of granulomatosis with polyangiitis in own material.
    Clin Exp Rheumatol 2018 May 4. Epub 2018 May 4.
    The Third Department of Pneumonology, National Research Institute of Tuberculosis and Lung Diseases, Warsaw, Poland.
    Objectives: Nasal carriage of Staphylococcus aureus and its superantigens (SAg) seem to be a risk factor disease exacerbation in granulomatosis with polyangiitis (GPA). We investigated the association between the presence of SAg in nasal swabs and activity of disease in GPA patients also taking into account correlation with an antimicrobial treatment.

    Methods: In a prospective study of a total of 150 GPA patients hospitalised in the period 2009-2016, nasal swabs were examined for the presence of Staphylococcus aureus and SAg. Read More

    Long-term risk and outcome of infection-related hospitalization in granulomatosis with polyangiitis: a nationwide population-based cohort study.
    Scand J Rheumatol 2018 May 9:1-6. Epub 2018 May 9.
    a Copenhagen Lupus and Vasculitis Clinic, Center for Rheumatology and Spine Diseases , Copenhagen University Hospital, Rigshospitalet , Copenhagen , Denmark.
    Objective: To assess the long-term risk and outcome of infection-related hospitalization (IH) among patients with granulomatosis with polyangiitis (GPA).

    Method: We used administrative databases to establish a GPA cohort (n = 398), construct a comparison cohort of population controls (n = 3980), and collect clinical data. Cox regression analyses were used to determine hazard ratios (HRs) as a measure of relative risk. Read More

    Rise and fall of the eosinophils in heart failure: a rare but important phenomenon seen with cardiomyopathy.
    BMJ Case Rep 2018 May 7;2018. Epub 2018 May 7.
    Cardiology, University Hospitals Coventry and Warwickshire, Coventry, UK.
    A 65-year-old lady and a 69-year-old gentleman, both with a background history of adult-onset asthma, presented with clinical features of heart failure (HF). High-sensitivity cardiac troponin T and eosinophils were significantly raised, along with poor left ventricular (LV) systolic function on cardiac imaging. Endocardial and skin biopsy (in cases 1 and 2, respectively) showed eosinophilic infiltration. Read More

    Primary Skull Base Lymphoma: Manifestations and Clinical Outcomes of a Great Imitator.
    Otolaryngol Head Neck Surg 2018 May 1:194599818773994. Epub 2018 May 1.
    2 Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA.
    Objectives Primary skull base lymphoma (PSBL) represents a rare manifestation of extranodal lymphoma. Presenting with nonspecific symptomatology and imaging findings, PSBL often masquerades as more common cranial base pathology and thus can present a diagnostic challenge. The objectives of this study were to characterize the manifestations and clinical outcomes of PSBL. Read More

    Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry.
    Autoimmun Rev 2018 May 3. Epub 2018 May 3.
    National Referral Centre for rare Juvenile Rheumatological and Autoimmune Diseases, Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, CNRS, Claude Bernard University Lyon 1, Lyon, France. Electronic address:
    Objective: The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

    Methods: Patients with SNV from the French Vasculitis Study Group registry were divided into 5 groups according to the date of diagnosis: <1980, 1980-1989, 1990-1999, 2000-2010, and ≥ 2010. The causes of death were classified as vasculitis, infection, cardiovascular, malignancy, miscellaneous, or unknown. Read More

    Combination Therapy With Rituximab and Cyclophosphamide for Remission Induction in ANCA Vasculitis.
    Kidney Int Rep 2018 Mar 14;3(2):394-402. Epub 2017 Nov 14.
    Division of Nephrology, Massachusetts General Hospital, Boston, Massachusetts, USA.
    Introduction: Remission induction in antineutrophil cytoplasmic autoantibody (ANCA) vasculitis may be complicated by slow response to treatment and toxicity from glucocorticoids. We describe outcomes with a novel remission induction regimen combining rituximab with a short course of low-dose, oral cyclophosphamide and an accelerated prednisone taper.

    Methods: Patients were included in this retrospective study if they had newly diagnosed or relapsing ANCA vasculitis with a Birmingham Vasculitis Activity Score for Wegener Granulomatosis (BVAS-WG) ≥3 and received a standardized remission induction regimen. Read More

    Metabolic pathways and immunometabolism in rare kidney diseases.
    Ann Rheum Dis 2018 May 3. Epub 2018 May 3.
    Vasculitis Clinical Research Consortium, Philadelphia, Pennsylvania, USA.
    Objectives: To characterise renal tissue metabolic pathway gene expression in different forms of glomerulonephritis.

    Methods: Patients with nephrotic syndrome (NS), antineutrophil cytoplasmic antibody-associated vasculitis (AAV), systemic lupus erythematosus (SLE) and healthy living donors (LD) were studied. Clinically indicated renal biopsies were obtained at time of diagnosis and microdissected into glomerular and tubulointerstitial compartments. Read More

    Long-term efficacy of remission-maintenance regimens for ANCA-associated vasculitides.
    Ann Rheum Dis 2018 May 3. Epub 2018 May 3.
    Department of Internal Medicine, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Paris, France.
    Objective: To compare long-term efficacy of remission-maintenance regimens in patients with newly diagnosed or relapsing antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides.

    Methods: The 28-month Maintenance of Remission using Rituximab in Systemic ANCA-associated Vasculitis trial compared rituximab with azathioprine to maintain remission in patients with newly diagnosed or relapsing granulomatosis with polyangiitis, microscopic polyangiitis or renal-limited ANCA-associated vasculitis. Thereafter, prospective patient follow-up lasted until month 60. Read More

    [Clinical Analysis of 40 Patients with Eosinophilic Lung Diseases in Peking Union Medical College Hospital].
    Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2018 Apr;40(2):170-177
    Department of Respiratory Medicine,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
    Objective To summarize the clinical features of eosinophilic lung diseases(ELD). Methods We retrospectively analyzed the clinical manifestations,laboratory findings,accessory examination results,and pathology of 40 patients who were diagnosed with ELD and hospitalized in Peking Union Medical College Hospital from January 2013 to December 2016.Results There were 19 males and 21 females,and the average age was(48. Read More

    Rituximab for induction and maintenance therapy of granulomatosis with polyangiitis: a single-centre cohort study on 114 patients.
    Rheumatology (Oxford) 2018 May 2. Epub 2018 May 2.
    National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Descartes, Paris, France.
    Objectives: To assess efficacy and safety of rituximab (RTX) induction and maintenance therapy for granulomatosis with polyangiitis (GPA) in a single-centre cohort study.

    Methods: All patients with active GPA, not enrolled in trials, who received ⩾1 RTX infusion(s) for induction were included. At remission, protocolized maintenance RTX infusions were given every 6 months for 18 months. Read More

    Pyoderma gangrenosum-like ulcerations in granulomatosis with polyangiitis: two cases and literature review.
    Rheumatol Int 2018 May 2. Epub 2018 May 2.
    Dermatology Unit, Department of Physiopathology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Pace 9, 20122, Milan, Italy.
    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing small vessel vasculitis associated with circulating anti-neutrophil cytoplasmic antibodies (ANCAs). Skin manifestations, mostly represented by palpable purpura, papulonodular lesions and livedo reticularis, are present in up to 50% of the cases. Ulcerations with undermined, raised erythematous-violaceous border resembling pyoderma gangrenosum (PG) have rarely been reported as skin involvement in GPA. Read More

    A 92-Year-Old Male with Eosinophilic Asthma Presenting with Recurrent Palpable Purpuric Plaques.
    Dermatopathology (Basel) 2018 Jan-Mar;5(1):44-48. Epub 2018 Mar 16.
    Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA.
    Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis affecting the small and medium-sized vasculature. It is commonly associated with asthma and eosinophilia. Most patients are diagnosed at around the age of 40. Read More

    Simultaneous Unilateral Presentation of Three Different Ocular Manifestations of Granulomatosis with Polyangiitis.
    J Ophthalmic Vis Res 2018 Apr-Jun;13(2):203-206
    Feiz Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
    Purpose: To report the simultaneous presentation of three ocular manifestations of granulomatosis with polyangiitis in one eye.

    Case Report: A 42-year-old man with a confirmed diagnosis of granulomatosis with polyangiitis was referred to the emergency room with sudden blurred vision. Eye examination showed hyperemic conjunctiva due to necrotizing scleritis in the superior nasal quadrant of the left eye, a mass in the left superior lid, as well as central retinal artery occlusion in the same eye. Read More

    Proteinase-3 and myeloperoxidase serotype in relation to demographic factors and geographic distribution in anti-neutrophil cytoplasmic antibody-associated glomerulonephritis.
    Nephrol Dial Transplant 2018 Apr 30. Epub 2018 Apr 30.
    Department of Nephrology and Medical and Health Sciences, Linköping University, Linköping, Sweden.
    Background: In anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis, antigen specificity varies between myeloperoxidase (MPO) and proteinase 3 (PR3). This has been reported to vary in relation to age, gender, geography and extrarenal manifestations. However, studies are difficult to compare as criteria for inclusion vary. Read More

    Severe localised granulomatosis with polyangiitis (Wegener's granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review.
    BMC Neurol 2018 May 1;18(1):59. Epub 2018 May 1.
    Department of Rheumatology, The Royal London and Mile End Hospitals, Barts Health NHS Trust, Bancroft Road, London, E1 4DG, UK.
    Background: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarely, pituitary infiltration.

    Case Presentation: We describe the case of a 32 year-old woman with limited but severe GPA manifesting as progressive cranial nerve palsies and pituitary dysfunction. Read More

    A Case of dilated Cardiomyopathy with Eosinophilic Granulomatosis with Polyangiitis in which Active Myocardial Inflammation was Only Detected by Endomyocardial Biopsy.
    Intern Med 2018 Apr 27. Epub 2018 Apr 27.
    Department of Cardio-Renal Medicine and Hypertension, Nagoya City University Graduate School of Medical Sciences, Japan.
    Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare type of systemic vasculitis. Cardiac involvement is the main cause of death in patients with this disease. We herein report a case of congestive heart failure in a patient with EGPA. Read More

    Methylprednisolone and plasmapheresis are effective for life-threatening diffuse alveolar hemorrhage and gastrointestinal hemorrhage in granulomatosis with polyangiitis: A case report and literature review.
    Medicine (Baltimore) 2018 Apr;97(17):e0592
    Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
    Rationale: The treatment of granulomatosis with polyangiitis (GPA) with life-threatening complications, such as diffuse alveolar hemorrhage (DAH) and gastrointestinal hemorrhage (GIH), remains challenging.

    Patient Concerns: A 70-year-old female presented with a 6-month history of a productive cough and a 10-day history of arthralgia that progressed to respiratory failure and massive hematochezia.

    Diagnoses: Chest high-resolution computed tomography (HRCT) revealed multiple nodules, masses, and cavities. Read More

    Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission: results of a multicentre, randomised controlled, phase III trial (MAINRITSAN2).
    Ann Rheum Dis 2018 Apr 25. Epub 2018 Apr 25.
    Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases: Vasculitis and Scleroderma, Cochin Hospital, Paris Descartes University, Paris, France.
    Objective: To compare individually tailored, based on trimestrial biological parameter monitoring, to fixed-schedule rituximab reinfusion for remission maintenance of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAVs).

    Methods: Patients with newly diagnosed or relapsing granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) in complete remission after induction therapy were included in an open-label, multicentre, randomised controlled trial. All tailored-arm patients received a 500 mg rituximab infusion at randomisation, with rituximab reinfusion only when CD19+B lymphocytes or ANCA had reappeared or ANCA titre rose markedly based on trimestrial testing until month 18. Read More

    Validation of the ANCA-associated vasculitis patient-reported outcomes (AAV-PRO) questionnaire.
    Ann Rheum Dis 2018 Apr 25. Epub 2018 Apr 25.
    Department of Rheumatology, University of Pennsylvania, Philadelphia, Massachusetts, USA.
    ObjectivesTo finalise and validate a disease-specific patient-reported outcome (PRO) measure: the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire. Using a 35-item candidate questionnaire developed following 50 qualitative interviews in the UK, USA and Canada, a longitudinal survey was conducted to determine the final scale structure and validate the AAV-PRO.

    Methods: Participants were recruited via Vasculitis UK and the Vasculitis Patient-Powered Research Network. Read More

    Circulating Cytokine Profiles and Antineutrophil Cytoplasmic Antibody Specificity in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.
    Arthritis Rheumatol 2018 Apr 25. Epub 2018 Apr 25.
    Boston University and VA Boston Healthcare System, Boston, Massachusetts.
    Objective: To evaluate circulating cytokine profiles in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), classified by antineutrophil cytoplasmic antibody (ANCA) specificity (proteinase 3 ANCA [PR3-ANCA] versus myeloperoxidase ANCA [MPO-ANCA]) or by clinical diagnosis (granulomatosis with polyangiitis [GPA] versus microscopic polyangiitis [MPA]).

    Methods: A panel of 29 cytokines was tested in 186 patients with active AAV at inclusion into the Rituximab in AAV trial. Cytokine concentrations were compared between groups within each classification system. Read More

    All-cause and Cause-specific Mortality in Granulomatosis with Polyangiitis: A Population-based Study.
    Arthritis Care Res (Hoboken) 2018 Apr 25. Epub 2018 Apr 25.
    Arthritis Research Canada, Vancouver, British Columbia, Canada.
    Objective: To investigate all-cause and cause-specific mortality in patients with newly diagnosed granulomatosis with polyangiitis (GPA) between two calendar time periods, 1997-2004 and 2005-2012.

    Methods: Using an administrative health database, we compared all incident cases of GPA to non-GPA controls matched based on sex, age and entry time. Study cohorts were divided into two sub-groups based on the year of diagnosis (i. Read More

    Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides.
    Front Immunol 2018 9;9:680. Epub 2018 Apr 9.
    Department of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany.
    Anti-neutrophil cytoplasmic autoantibodies (ANCA) targeting proteinase 3 (PR3) and myeloperoxidase expressed by innate immune cells (neutrophils and monocytes) are salient diagnostic and pathogenic features of small vessel vasculitis, comprising granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic GPA. Genetic studies suggest that ANCA-associated vasculitides (AAV) constitute separate diseases, which share common immunological and pathological features, but are otherwise heterogeneous. The successful therapeutic use of anti-CD20 antibodies emphasizes the prominent role of ANCA and possibly other autoantibodies in the pathogenesis of AAV. Read More

    Targeting the Interleukin-5 Pathway for Treatment of Eosinophilic Conditions Other than Asthma.
    Front Med (Lausanne) 2018 6;5:49. Epub 2018 Apr 6.
    Hôpital Erasme, Department of Internal Medicine, Université Libre de Bruxelles, Brussels, Belgium.
    Improved understanding of the contribution of eosinophils to various chronic inflammatory conditions, most notably allergic asthma, has encouraged development of monoclonal antibodies specifically targeting mediators and surface receptors involved in eosinophil expansion and activation. The pivotal role of interleukin-5 (IL-5) in eosinophil biology, its high specificity for this leukocyte subset, and its involvement in the majority of eosinophilic conditions make it a very enticing target for treatment of eosinophil-mediated disorders. Two types of antibodies have been developed to target eosinophils: antibodies against IL-5 (mepolizumab and reslizumab), and an antibody against the IL-5-receptor-alpha-chain (IL-5Rα) (benralizumab). Read More

    Granulomatosis with Polyangiitis Localized in the Greater Omentum.
    Case Rep Surg 2018 28;2018:6145903. Epub 2018 Feb 28.
    Department of Pathology, Mitsui Memorial Hospital, Tokyo, Japan.
    Granulomatosis with polyangiitis (GPA) is known as anti-neutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis and typically manifests as pulmonary-renal syndrome, but the disease is not limited to pulmonary or renal systems. The inflammation can involve whole body organs. In addition, the ANCA titer does not always become positive. Read More

    Smoking Is a Risk Factor for Relapse of Antimyeloperoxidase Antibodies-Associated Vasculitis.
    J Clin Rheumatol 2018 Apr 5. Epub 2018 Apr 5.
    Background/objective: Several studies have identified predictors of relapse in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. However, the role of smoking as a risk factor of relapse has not been elucidated. Therefore, this study aimed to evaluate whether a history of smoking is a dose-dependent independent risk factor for antineutrophil cytoplasmic antibody-associated small-vessel vasculitis relapse. Read More

    A patient-centred multi-domain instrument for improving the clarity of outcomes reporting and documentation in complex airway surgery.
    Clin Otolaryngol 2018 Apr 17. Epub 2018 Apr 17.
    The Robert White Centre for Airway, Voice& Swallowing, Department of Ear Nose and Throat Surgery, Poole Hospital NHS Foundation Trust, Longfleet Road, Poole, BH15 2JB, United Kingdom.
    Laryngotracheal stenosis refers to abnormal narrowing of the central airways from larynx to carina. It is caused by an eclectic group of conditions that include intubation-related airway stenosis, granulomatosis with polyangiitis, tracheal compression or malignancy, and congenital laryngotracheal malformations. A significant proportion of patients with this condition can be satisfactorily treated using a combination of endoscopic and open surgical techniques and when definitive treatment is not possible, most of the remaining patients can be managed with a combination of endoscopic airway maintenance, stents, T-tubes, and/or tracheostomies. Read More

    Computed Tomography (CT) Angiography in Pre-Embolization Assessment of Location of Gastrointestinal Bleeding in Paediatric Patient with Granulomatosis with Polyangiitis (Wegener's Granulomatosis) - Case Report.
    Pol J Radiol 2017 20;82:589-592. Epub 2017 Oct 20.
    Department of Paediatric Radiology, Medical University of Warsaw, Warsaw, Poland.
    Background: Acute gastrointestinal bleeding is an emergency with a high morbidity and mortality. Early diagnosis and appropriate intervention can be lifesaving and can prevent long-term complications.

    Case Report: In this case report, we discuss and illustrate the role of CT angiography in the evaluation of acute, active gastrointestinal haemorrhage and show its usefulness prior to embolization. Read More

    A clinical threat in patients with granulomatosis polyangiitis in remission: Subglottic stenosis.
    Eur J Rheumatol 2018 Mar 11;5(1):69-71. Epub 2017 Apr 11.
    Division of Rheumatology, Department of Internal Medicine, Süleyman Demirel University School of Medicine, Isparta, Turkey.
    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatous disease that involves small- and medium-sized arteries and affects the main respiratory tracts and kidneys. Upper respiratory tract involvement usually occurs in 90% of patients, who most frequently present with symptoms of chronic sinusitis. Subglottic stenosis (SS) is a rare and severe complication that is usually observed in approximately 15% of patients. Read More

    Overall Disability Sum Score for Clinical Assessment of Neurological Involvement in Eosinophilic Granulomatosis With Polyangiitis.
    J Clin Rheumatol 2018 Jun;24(4):197-202
    From the Operative Unit of Rheumatology and.
    Aim: The aim of this study was to verify the application of Overall Disability Sum Score (ODSS) for standardized clinical assessment of neurological involvement in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and its correlation with treatment response and long-term outcomes.

    Methods: Consecutive EGPA patients referred to our tertiary vasculitis center were retrospectively evaluated. Patients' neurological damage and disability were systematically assessed with Vasculitis Damage Index and ODSS. Read More

    ANCA associated vasculitis in patients from Saudi Arabia.
    Pak J Med Sci 2018 Jan-Feb;34(1):88-93
    Najma Khalil, M.Sc., M.Phil, Division of Rheumatology, College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
    Objective: To explore clinical and laboratory features, therapy and outcome of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) patients from our tertiary care center.

    Methods: This study of AAV patients seen in Rheumatology clinics at King Khalid University hospital (KKUH), King Saud University, Riyadh during the period 1990-2014 was carried out retrospectively. Demographic, clinical, haematological and immunological parameters along with therapy, complications and outcome were retrieved from patients' medical charts. Read More

    Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis.
    Drugs Today (Barc) 2018 Feb;54(2):93-101
    Royal Brompton Hospital, London, UK.
    Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare but potentially life-threatening antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis which affects, to varying degrees, the lungs, paranasal sinuses, heart, kidneys, skin and peripheral nervous system. It is strongly associated with asthma. Peripheral eosinophilia is a defining feature of EGPA and eosinophilic inflammation is often observed in biopsies of affected tissues. Read More

    Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis.
    Ophthalmic Res 2018 Apr 10. Epub 2018 Apr 10.
    Massachusetts Eye Research and Surgery Institute, Waltham, Massachusetts, USA.
    Purpose: Vision-threatening ocular inflammation can be a devastating complication of granulomatosis with polyangiitis (GPA). Here we performed a retrospective observational study to describe the safety and efficacy of treating scleritis and uveitis with either cyclophosphamide or rituximab in GPA.

    Methods: A chart review of patients diagnosed with GPA-associated scleritis or uveitis, treated with either cyclophosphamide or rituximab as the final therapy at our clinic, was conducted. Read More

    Do pulmonary findings of granulomatosis with polyangiitis respond to anti-tuberculosis treatment?
    Rheumatol Int 2018 Jun 9;38(6):1131-1138. Epub 2018 Apr 9.
    Division of Rheumatology, Department of Internal Medicine, School of Medicine, Eskişehir Osmangazi University, 26480, Eskisehir, Turkey.
    Granulomatosis with polyangiitis (GPA) involves upper and lower respiratory tracts and kidneys. Lung involvement is among the most important organ involvements in GPA. GPA's lung involvement might be confused with other granulomatous conditions with lung involvement. Read More

    Tuberculosis and Other Granulomatous Diseases of the Airway.
    Thorac Surg Clin 2018 May;28(2):155-161
    Laval University, 6364, Chemin Royal, Saint-Laurent-de-l'Île-d'Orléans, Quebec City, Québec G0A3Z0, Canada.
    Granulomatous diseases of the airway are challenging lesions to diagnose and effectively manage not only because they are uncommon but also because they can occur in different forms, each with unique clinical and radiological characteristics. Most such lesions can be effectively managed conservatively with repeated airway dilatation, use of intraluminal stents, and specific antimicrobial treatment. The only exception is those lesions presenting with localized airway obstruction wherein surgical resection may be indicated and beneficial. Read More

    Gastrointestinal Tract Vasculopathy: Clinicopathology and Description of a Possible "New Entity" With Protean Features.
    Am J Surg Pathol 2018 Apr 5. Epub 2018 Apr 5.
    Department of Pathology, Stanford University School of Medicine, Stanford, CA.
    Noninfectious gastrointestinal (GI) vasculopathic disorders are rare and are often overlooked in histopathologic examination or when forming differential diagnoses due to their rarity. However, involvement of the GI tract may lead to serious complications, including ischemia and perforation. Since awareness of the types of vasculopathy that may involve the GI tract is central to arriving at a correct diagnosis, we reviewed our institutional experience with GI tract vasculopathy in order to enhance diagnostic accuracy of these rare lesions. Read More

    Hypocomplementemia is associated with worse renal survival in ANCA-positive granulomatosis with polyangiitis and microscopic polyangiitis.
    PLoS One 2018 5;13(4):e0195680. Epub 2018 Apr 5.
    Department of Internal Medicine, CHU de Caen, Caen, France.
    Recent data suggest the existence of a complement alternative pathway activation in the pathogenesis of antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV), a condition that remains poorly understood. This study aims to assess the clinical characteristics and outcomes of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) patients with regard to their plasma complement levels at diagnosis. A retrospective monocentric study carried out at Caen University Hospital led to the identification of proteinase-3- or myeloperoxidase-ANCA-positive GPA and MPA patients from January 2000 to June 2016 and from September 2011 to June 2016, respectively. Read More

    Trimethoprim-sulfamethoxazole and antineutrophil cytoplasmic antibodies-associated vasculitis.
    Curr Opin Rheumatol 2018 Apr 4. Epub 2018 Apr 4.
    Division of Rheumatology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.
    Purpose Of Review: In antineutrophil cytoplasmic antibodies-associated vasculitis (AAV), the treatment of choice is either Rituximab or cyclophosphamide in combination with steroids. In less extended forms of AAV, however, less toxic regimens are being used.In the current review, we will focus on the role of one of these less toxic regimens, that is trimethoprim-sulfamethoxazole monotherapy as induction treatment or as maintenance therapy in AAV. Read More

    Granulomatosis With Polyangiitis as an Uncommon Cause of Panhypopituitarism.
    J Clin Rheumatol 2018 Mar 20. Epub 2018 Mar 20.
    Division of Endocrinology and Metabolism Department of Internal Medicine Acibadem University School of Medicine Istanbul, Turkey Division of Rheumatology Department of Internal Medicine Acibadem University School of Medicine Istanbul, Turkey Department of Ophthalmology Acibadem University School of Medicine Istanbul, Turkey Department of Neurosurgery Kocaeli University School of Medicine Pituitary Research Center Kocaeli, Turkey.

    New biologics for allergic diseases.
    Expert Rev Clin Immunol 2018 Apr 10;14(4):285-296. Epub 2018 Apr 10.
    b The Cohen Family Asthma Institute, Division of Pulmonary, Critical Care, and Sleep Medicine , National Jewish Health , Denver , CO , USA.
    Introduction: Allergic conditions such as asthma and atopic dermatitis have a high prevalence but represent a heterogeneous group of diseases despite similar clinical presentation and underlying pathophysiology. A better understanding of the phenotypes and endotypes of these diseases has driven rapid development of biologic medications targeting many steps of the inflammatory pathways. Areas covered: There are 2 major inflammatory pathways that drive allergic diseases: Type-2 (Th-2) inflammation and non-type 2 inflammation. Read More

    Delta Neutrophil Index Is Associated with Vasculitis Activity and Risk of Relapse in ANCA-Associated Vasculitis.
    Yonsei Med J 2018 May;59(3):397-405
    Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
    Purpose: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV).

    Materials And Methods: We reviewed the medical records of 97 patients having DNI results. Read More

    Eosinophilic Granulomatosis With Polyangiitis: Newer Therapies.
    Curr Rheumatol Rep 2018 Apr 2;20(5):23. Epub 2018 Apr 2.
    GIRAT: Grupo de Investigación en Reumatología, Autoinmunidad y Medicina Traslacional, Fundación Valle del Lili, Universidad Icesi, Cl 18 #, Cali, 122-135, Colombia.
    Purpose Of Review: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic disseminated vasculitis associated with extravascular granulomas in patients suffering from asthma and tissue eosinophilia. Current therapies to achieve remission and prevent relapse include glucocorticoids and immunosuppressants like cyclophosphamide.

    Recent Findings: With the right treatment, clinical prognosis is favorable, so concerted efforts have been made in recent years to find new alternatives for treating severe EGPA. Read More

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