Interstitial lung diseases associated with ANCA positivity: A different disease spectrum from interstitial pneumonia with autoimmune features.

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  • Additional Information
    • Source:
      Publisher: Elsevier Masson SAS Country of Publication: France NLM ID: 101746324 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2590-0412 (Electronic) Linking ISSN: 25900412 NLM ISO Abbreviation: Respir Med Res Subsets: MEDLINE
    • Publication Information:
      Original Publication: [Issy-les-Moulineaux] : Elsevier Masson SAS, [2019]-
    • Subject Terms:
      Antibodies, Antineutrophil Cytoplasmic*/blood ; Antibodies, Antineutrophil Cytoplasmic*/immunology ; Lung Diseases, Interstitial*/immunology ; Lung Diseases, Interstitial*/etiology ; Lung Diseases, Interstitial*/diagnosis; Humans ; Male ; Female ; Retrospective Studies ; Middle Aged ; Aged ; Idiopathic Interstitial Pneumonias/diagnosis ; Idiopathic Interstitial Pneumonias/complications ; Idiopathic Interstitial Pneumonias/physiopathology ; Idiopathic Interstitial Pneumonias/immunology ; Immunosuppressive Agents/therapeutic use ; Respiratory Function Tests ; Autoimmune Diseases/immunology ; Autoimmune Diseases/complications ; Autoimmune Diseases/diagnosis ; Autoimmune Diseases/blood ; Glucocorticoids/therapeutic use
    • Abstract:
      Background: Anti-neutrophil cytoplasmic antibody (ANCA) is a type of autoantibodies associated with vasculitis. ANCA positivity is commonly observed in interstitial lung disease (ILD) patients. 7%-10% of ANCA-positive ILD patients don't present any symptoms of systemic vasculitis and are termed ANCA-positive idiopathic interstitial pneumonia (ANCA-IIP). Some researchers propose that ANCA-IIP should be categorized as interstitial pneumonia with autoimmune features (IPAF), although the official ATS/ERS statements exclude ANCA-IIP from this classification. Whether ANCA-IIP should be categorized into the entity of IPAF is still debatable.
      Methods: Patients diagnosed with ANCA-IIP and those with IPAF were analyzed in a retrospective study of ILD. The clinical outcomes were determined through pulmonary function tests (PFTs) after a one-year follow-up, as well as assessing all-cause mortality.
      Results: 27 patients with ANCA-IIP and 143 patients with IPAF were analyzed from a cohort of 995 patients with ILD. Patients in the ANCA-IIP group had an older age and a high proportion of males compared to those in the IPAF group. PFT results at baseline were similar between the two groups, except for a better FEV1% in the ANCA-IIP group. Glucocorticoid and immunosuppressive therapy improved pulmonary function in patients with IPAF, but it continued to deteriorate after one year of treatment in the ANCA-IIP group. Furthermore, the all-cause mortality rate was significantly higher in the ANCA-IIP group than in the IPAF group (22.2% vs. 6.3%, P = 0.017).
      Conclusion: The responses to glucocorticoid and immunosuppressive therapy differ between the ANCA-IIP and IPAF groups, leading to divergent prognoses. Therefore, it is inappropriate to classify ANCA-IIP as part of IPAF.
      Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
      (Copyright © 2024 SPLF and Elsevier Masson SAS. All rights reserved.)
    • Contributed Indexing:
      Keywords: ANCA; Interstitial lung disease; Interstitial pneumonia with autoimmune features
    • Accession Number:
      0 (Antibodies, Antineutrophil Cytoplasmic)
      0 (Immunosuppressive Agents)
      0 (Glucocorticoids)
    • Publication Date:
      Date Created: 20240608 Date Completed: 20241216 Latest Revision: 20241216
    • Publication Date:
      20241217
    • Accession Number:
      10.1016/j.resmer.2024.101111
    • Accession Number:
      38851036