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Background <p>Patients with anti-neutrophil cytoplasmic antibody (ANCA)-positive interstitial lung disease (ILD) but without evidence of systemic vasculitis have been reported in studies and are classified as isolated ANCA-positive idiopathic interstitial pneumonia (IIP). However, the clinical significance of ANCA, particularly myeloperoxidase (MPO) -ANCA in IIP remains poorly understood. This study aims to investigate the differences between ANCA-positive and ANCA-negative IIP patients and further explore the impact of MPO-ANCA on clinical manifestations and prognostic outcomes.</p> Methods <p>We reviewed 408 ILD patients with available ANCA results from January 2012 to September 2021. 61 patients diagnosed with microscopic polyangiitis-associated ILD were not included in the analysis. A comparative analysis was performed between 61 isolated ANCA-positive IIP patients (ANCA-IIP group) and 286 ANCA-negative IIP patients (IIP group). We further conducted subgroup analyses based on the status of MPO-ANCA.</p> Results <p>Baseline clinical characteristics, pulmonary function tests, radiological features and all-cause mortality were similar between ANCA-IIP and IIP groups. When comparing the MPO-ANCA-IIP group with the IIP group and the non-MPO-ANCA-IIP group separately, a higher proportion of fibrotic features was observed on imaging (<i>P</i> = 0.004 vs IIP group; <i>P</i> = 0.031 vs non-MPO-ANCA-IIP group). After one year of treatment, the MPO-ANCA-IIP group showed a significantly greater decline in pulmonary function parameters compared to both the IIP group and the non-MPO-ANCA-IIP group. The frequency of pulmonary function decline was significantly higher in the MPO-ANCA-IIP group compared to the non-MPO-ANCA-IIP group (<i>P</i> = 0.026). Additionally, MPO-ANCA was not found to be statistically associated with mortality among patients with IIP.</p> Conclusion <p>ANCA-IIP patients had similar clinical characteristics and prognoses with IIP patients. MPO-ANCA-IIP patients had more prominent fibrosis on imaging and a greater decline in pulmonary function following treatment. Special attention should be paid to MPO-ANCA positivity during the diagnosis and treatment of IIP patients.</p> Trial registration <p>ClinicalTrials.gov: NCT04413149, May 2020.</p>

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Clinical significance of anti-neutrophil cytoplasmic antibody in idiopathic interstitial pneumonia: a retrospective observational study

  • 期刊论文

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  • 发表日期：

    2025年5月29日

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  • Xin Sun,
  • Wenyan Zhu,
  • Chunsheng Zhou,
  • Peijun Xue,
  • Zongru Li,
  • Weihong Zhang,
  • Jiuliang Zhao,
  • Ting Zhang,
  • Min Peng,
  • Juhong Shi,
  • Chen Wang

摘要

Background

Patients with anti-neutrophil cytoplasmic antibody (ANCA)-positive interstitial lung disease (ILD) but without evidence of systemic vasculitis have been reported in studies and are classified as isolated ANCA-positive idiopathic interstitial pneumonia (IIP). However, the clinical significance of ANCA, particularly myeloperoxidase (MPO) -ANCA in IIP remains poorly understood. This study aims to investigate the differences between ANCA-positive and ANCA-negative IIP patients and further explore the impact of MPO-ANCA on clinical manifestations and prognostic outcomes.

Methods

We reviewed 408 ILD patients with available ANCA results from January 2012 to September 2021. 61 patients diagnosed with microscopic polyangiitis-associated ILD were not included in the analysis. A comparative analysis was performed between 61 isolated ANCA-positive IIP patients (ANCA-IIP group) and 286 ANCA-negative IIP patients (IIP group). We further conducted subgroup analyses based on the status of MPO-ANCA.

Results

Baseline clinical characteristics, pulmonary function tests, radiological features and all-cause mortality were similar between ANCA-IIP and IIP groups. When comparing the MPO-ANCA-IIP group with the IIP group and the non-MPO-ANCA-IIP group separately, a higher proportion of fibrotic features was observed on imaging (P = 0.004 vs IIP group; P = 0.031 vs non-MPO-ANCA-IIP group). After one year of treatment, the MPO-ANCA-IIP group showed a significantly greater decline in pulmonary function parameters compared to both the IIP group and the non-MPO-ANCA-IIP group. The frequency of pulmonary function decline was significantly higher in the MPO-ANCA-IIP group compared to the non-MPO-ANCA-IIP group (P = 0.026). Additionally, MPO-ANCA was not found to be statistically associated with mortality among patients with IIP.

Conclusion

ANCA-IIP patients had similar clinical characteristics and prognoses with IIP patients. MPO-ANCA-IIP patients had more prominent fibrosis on imaging and a greater decline in pulmonary function following treatment. Special attention should be paid to MPO-ANCA positivity during the diagnosis and treatment of IIP patients.

Trial registration

ClinicalTrials.gov: NCT04413149, May 2020.