Diagnosis and management of ANCA associated vasculitis

  title={Diagnosis and management of ANCA associated vasculitis},
  author={Annelies E. Berden and Arda G{\"o}çeroğlu and David R. W. Jayne and Raashid A Luqmani and Niels Rasmussen and Jan A. Bruijn and Ingeborg M. Bajema},
  journal={BMJ : British Medical Journal},
#### Summary points Vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) are systemic autoimmune diseases of unknown cause that affect small to medium sized blood vessels. They include granulomatosis with polyangiitis (formerly Wegener’s granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). This review mainly focuses on granulomatosis with polyangiitis and microscopic polyangiitis. Although they are… 
Biological drugs in ANCA-associated vasculitis.
Granuloma in ANCA-Associated Vasculitides: Another Reason to Distinguish Between Syndromes?
It is hypothesized that, as for ANCA, necrotizing granulomatous inflammation and its clinical manifestations are discriminators, assisting subclassification of AAV and/or GPA subphenotypes which will be useful both for designing clinical trials and for treating patients successfully.
Granulomatosis with polyangiitis: update and key concepts
Key aspects of granulomatosis with polyangiitis are reviewed, including aspects such as epidemiology, pathophysiology, clinical manifestations, diagnosis and treatment, which are fundamental to the development of new therapies for disease management.
Can genetic studies predict treatment response in antineutrophil cytoplasmic antibody‐associated vasculitis?
  • M. Harigai
  • Biology, Medicine
    International journal of rheumatic diseases
  • 2019
Genetic markers of AAV have been extensively studied using both genome-wide and candidate-gene approaches, and are providing clues for better understanding the pathogenesis of A AV and for developing targeted therapies.
ANCA-associated vasculitis and malignancy: current evidence for cause and consequence relationships.
The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age
A female teenager presented with a history of subglottic stenosis diagnosed at the age of 12 and a presumptive diagnosis of ANCA-positive vasculitis was made, and she was started corticotherapy, cyclophosphamide, and plasmapheresis.
Clinically-Irrelevant Positivity for Serum Proteinase 3-ANCA in HIV Disease
Although ANCA detection may be helpful in diagnosing small-vessel vasculitis in the appropriate clinical scenario, screening for HIV infection should sometimes be considered to discriminate clinically irrelevant serum ANCA positivity.
Propylthiouracil-induced anti-neutrophil cytoplasmic antibody-associated vasculitis mimicking Kawasaki disease
This case demonstrates that children with PTU-associated AAV may present with clinical features mimicking Kawasaki disease, and that IVIG along with corticosteroid therapy may be effective in treating patients with drug-induced severe systemic AAV.
ANCA-Associated Vasculitis—ENT Involvement
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are idiopathic, immunologically mediated diseases predominantly affecting small vessels throughout the body (capillaries,


Long-term patient survival in ANCA-associated vasculitis
Patients with ANCA-associated vasculitis treated with conventional regimens are at increased risk of death compared with an age- and sex-matched population.
Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study.
  • A. Booth, M. Almond, D. Jayne
  • Medicine
    American journal of kidney diseases : the official journal of the National Kidney Foundation
  • 2003
A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies.
In patients with generalized vasculitis, the withdrawal of cyclophosphamide and the substitution of azathioprine after remission did not increase the rate of relapse, suggesting the duration of exposure to cycloph phosphamide may be safely reduced.
Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.
Investigation of prognostic value of clinical, laboratory, and pathologic features at the time of presentation on patient and renal survival in patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated microscopic polyangiitis and glomerulonephritis found pathology indices such as glomerular necrosis, glomersular crescents,glomerular sclerosis, and interstitial sclerosis were not predictive of renal survival.
Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis: follow-up data from European Vasculitis Study Group clinical trials
Cancer rates for AAV patients treated with conventional immunosuppressive therapy exceeded those expected for the general population, largely driven by an increased incidence of NMSC.
Association of Chronic Nasal Carriage of Staphylococcus aureus and Higher Relapse Rates in Wegener Granulomatosis
The possible beneficial influence of cotrimoxazole in Wegener granulomatosis, patients receiving prolonged continuous treatment with antibiotics, is examined, with special emphasis on current or recent use of antibiotics.
Outcome in glomerulonephritis due to systemic small vessel vasculitis: effect of functional status and non-vasculitic co-morbidity.
  • M. Little, L. Nazar, K. Farrington
  • Medicine
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2004
Non-vasculitic factors, particularly functional status as indicated by the Karnofsky performance score, play a major role in determining prognosis in SVV with renal involvement and should be an integral component of the decision making process when planning therapy, and in comparing outcomes between centres.
Does a gating policy for ANCA overlook patients with ANCA associated vasculitis? An audit of 263 patients
Adherence to a gating policy for ANCA testing coupled with close liaison between clinician and laboratory does not result in either a missed or delayed diagnosis of small-vessel vasculitis belonging to the WG-MPA spectrum.
Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis.
A clinical index of disease activity is devised, its use in several forms of necrotizing vasculitis is evaluated, and the weighted score is based on symptoms and signs in nine separate organ systems.
Renal vasculitis in Japan and the UK--are there differences in epidemiology and clinical phenotype?
  • R. Watts, D. Scott, H. Hashimoto
  • Medicine
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2008
Comparing the incidence of renal vasculitis in the UK with recent data from a Japanese population found Wegener's granulomatosis seems to be much less common in Japan than the UK, and microscopic polyangiitis predominating in Japan.