ANCA in systemic sclerosis, when vasculitis overlaps with vasculopathy: a devastating combination of pathologies.

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  • Additional Information
    • Source:
      Publisher: Oxford University Press Country of Publication: England NLM ID: 100883501 Publication Model: Print Cited Medium: Internet ISSN: 1462-0332 (Electronic) Linking ISSN: 14620324 NLM ISO Abbreviation: Rheumatology (Oxford) Subsets: MEDLINE
    • Publication Information:
      Original Publication: Oxford, UK : Avenel, N.J. : Oxford University Press ; Distributed by Mercury International, c1999-
    • Subject Terms:
    • Abstract:
      In patients with SSc, the coexistence of ANCA-associated vasculitis (SSc-AAV) has been reported to be associated with a severe disease course, including significant pulmonary and renal involvement. The presence of ANCA is not uncommon in patients with SSc, and therefore clinicians must maintain a high index of clinical suspicion about SSc-AAV. p-ANCA and anti-myeloperoxidase antibodies are the most common antibodies observed. Patients typically present with clinical features of microscopic polyangiitis or renal-limited vasculitis. There are multiple areas of potential interaction in the pathogenesis of SSc and AAV, which can exacerbate/compound vascular disease. In addition, similar patterns of major internal organ involvement (e.g. lung and kidneys) are seen in both conditions. We highlight a diagnostic approach to SSc-AAV and the paucity of data to inform management. As such, SSc-AAV is typically treated as per isolated AAV, which can potentially be hazardous in patients with SSc (e.g. due to the association between high-dose steroid and scleroderma renal crisis). We propose that this rare clinical entity warrants rigorous investigation, including definition of a therapeutic strategy to ameliorate the potentially devastating combination of pathologies in SSc-AAV.
      (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].)
    • Comments:
      Comment in: Rheumatology (Oxford). 2021 Nov 3;60(11):e407-e409. (PMID: 34213551)
    • Grant Information:
      National Institute for Health Research; NIHR; Imperial Biomedical Research Centre
    • Contributed Indexing:
      Keywords: ANCA; scleroderma; systemic sclerosis; vasculitis; vasculopathy
    • Accession Number:
      0 (Antibodies, Antineutrophil Cytoplasmic)
    • Publication Date:
      Date Created: 20210321 Date Completed: 20220103 Latest Revision: 20220103
    • Publication Date:
      20240829
    • Accession Number:
      10.1093/rheumatology/keab278
    • Accession Number:
      33744916