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3 results

  • Chapter 1.2 - Chapter

  • from 1 - Inflammatory Conditions
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book:
    Rare Causes of Stroke
    Published online:
    06 October 2022
    Print publication:
    01 September 2022, pp 34-39

  • Summary

    A 16 years old female patient admitted to hospital due to fatigue, myalgia, weight loss and night sweats lasting for 10 days. On examination, patient appeared pale with livedo reticularis on lower extremities. On the 2nd day of hospitalization, she developed acute right hemiparesis, dysarthria and right facial droop. Cranial computerized tomography was performed, and it revealed multiple bilateral lesions consistent with acute intracranial hemorrhage. The cranial MRI showed restricted diffusion in the left internal capsule which was in concordance with clinical signs of acute stroke. Past medical history of the patient revealed recurrent attacks of bronchitis. In the laboratory examination, patient had elevated acute phase markers, leukocytosis, mild anemia, thrombocytosis. Further investigations revealed significantly high c-ANCA and proteinase 3 antibody titers, whereas myeloperoxidase antibody was negative. High-resolution computerized tomography revealed lesions in both lungs. A diagnosis of Granulomatosis with Polyangiitis (GPA) was confirmed. high doses of glucocorticoids (30 mg/kg/day for the consecutive 5 days) was given as an induction part of treatment together with pulse dose of cyclophosphamide (1 gr/m2/month for the consecutive 6 months). The rituximab was added to treatment (375 mg/m2/week for the consecutive 4 weeks) since the patient was considered to have life-threating complication of underlying disease. The patient responded promptly with regression of neurological findings, decline in acute phase markers and significantly improvement in patient’s general condition

  • Morbidity and mortality associated with subglottic laryngotracheal stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): a single-centre experience in the United Kingdom

  • N P Jordan, H Verma, A Siddiqui, G A Morrison, D P D'Cruz
  • Journal:
    The Journal of Laryngology & Otology / Volume 128 / Issue 9 / September 2014
    Published online by Cambridge University Press:
    02 September 2014, pp. 831-837
    Print publication:
    September 2014
  • Objectives:

    We aimed to determine the prevalence of symptomatic subglottic laryngotracheal stenosis in patients with granulomatosis with polyangiitis (Wegener's granulomatosis); we also wanted to characterise the clinical outcomes and surgical interventions required, and the relapse rate in our cohort.

    Methods:

    We undertook a retrospective clinical review of all granulomatosis with polyangiitis patients with symptomatic subglottic laryngotracheal stenosis attending St Thomas' Hospital, London, United Kingdom.

    Results:

    Symptomatic subglottic laryngotracheal stenosis developed in 16 per cent of granulomatosis with polyangiitis patients attending our clinic. The median age of patients at diagnosis was 44 years (range: 34–81 years); 78 per cent of those presenting with subglottic laryngotracheal stenosis were women and 22 per cent were men. All patients were white; 67 per cent of patients were proteinase 3-antineutrophil cytoplasmic antibody-positive and 67 per cent developed relapsing disease requiring repeated surgical intervention. Subglottic laryngotracheal stenosis relapse was not associated with active systemic vasculitis elsewhere.

    Conclusion:

    Subglottic laryngotracheal stenosis is an uncommon but significant complication of granulomatosis with polyangiitis. Management of subglottic laryngotracheal stenosis requires a multi-disciplinary approach, with both rheumatological and otolaryngological expertise involved, given the relapsing nature of the disease.

  • Presentation and initial assessment of ENT problems in patients with granulomatosis with polyangiitis (Wegener's)

  • M Martinez Del Pero, D McKiernan, P Jani
  • Journal:
    The Journal of Laryngology & Otology / Volume 128 / Issue 8 / August 2014
    Published online by Cambridge University Press:
    22 July 2014, pp. 730-737
    Print publication:
    August 2014
  • Background:

    Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) is a primary systemic vasculitis that affects medium to small sized vessels throughout the body. It often presents with symptoms and signs involving the ear, nose, and head and neck area.

    Objective:

    To highlight salient features of ENT-related issues in granulomatosis with polyangiitis, and raise awareness of the condition.

    Methods:

    A case report of a patient with limited disease and an insidious onset is presented, along with a review of the current literature. In addition, basic initial management is described.

    Results:

    Eighty-six publications were used to describe salient features of ENT-related issues in granulomatosis with polyangiitis.

    Conclusion:

    The time to diagnosis has not reduced significantly in the last 10 years in the UK. A high index of suspicion is required for an earlier diagnosis of granulomatosis with polyangiitis.