Clinical significance of anti-neutrophil cytoplasmic antibody in idiopathic interstitial pneumonia: a retrospective observational study
Abstract 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...
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| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2025-05-01
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| Series: | BMC Pulmonary Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12890-025-03736-4 |
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| Summary: | Abstract 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. |
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| ISSN: | 1471-2466 |