Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease
Abstract Background Patients with rheumatoid arthritis (RA) or rheumatoid arthritis-associated interstitial lung disease (RA-ILD) are commonly positive for antineutrophil cytoplasmic antibodies (ANCAs). The causal relationship between RA-ILD and ANCAs and the role of ANCAs in RA-ILD remain unclear....
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2025-04-01
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| Online Access: | https://doi.org/10.1186/s12890-025-03644-7 |
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| author | Zhe Huang Tingting Wu Rongdan Lu Haijun Zhou Yun Zhang Li Huang Yongxiong Gan Hequn He |
| author_facet | Zhe Huang Tingting Wu Rongdan Lu Haijun Zhou Yun Zhang Li Huang Yongxiong Gan Hequn He |
| author_sort | Zhe Huang |
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| description | Abstract Background Patients with rheumatoid arthritis (RA) or rheumatoid arthritis-associated interstitial lung disease (RA-ILD) are commonly positive for antineutrophil cytoplasmic antibodies (ANCAs). The causal relationship between RA-ILD and ANCAs and the role of ANCAs in RA-ILD remain unclear. The purpose of this study was to estimate the prevalence of ANCAs in RA-ILD patients and to investigate the clinical characteristics and outcomes of ANCA-positive RA-ILD patients. Methods Data from 104 RA-ILD patients with available ANCA results at our centre from March 2010 to June 2024 were retrospectively reviewed. ANCA positivity was defined as the presence of any one or a combination of perinuclear ANCAs (P-ANCAs), cytoplasmic ANCAs (C-ANCAs), anti-MPO or anti-PR-3. Clinical data from each patient’s initial diagnosis were collected and analysed. The clinical characteristics and survival of the ANCA-positive and ANCA-negative RA-ILD groups were compared. Results Thirty-three out of the 104 (31.7%) RA-ILD patients were positive for ANCAs. The percentages of patients positive for P-ANCAs, MPO-ANCAs, C-ANCAs, and PR3-ANCAs were 27.9% (29/104), 1.9% (2/104), 1.9% (2/104), and 1.0% (1/104), respectively. Compared with ANCA-negative RA-ILD patients, a greater proportion of ANCA-positive RA-ILD patients had respiratory symptoms, serum autoantibody (ANA) positivity, poorer baseline pulmonary function, and acute exacerbations of ILD (AE-ILD). The usual interstitial pneumonia (UIP) pattern (57.6%) was the most common chest high-resolution computed tomography (HRCT) pattern observed. ANCA-positive RA-ILD patients were more likely to have traction bronchiectasis (P = 0.029), honeycombing (P < 0.001), and oddly shaped cysts (P = 0.020) than were ANCA-negative RA-ILD patients. Univariate Cox analysis revealed that P-ANCA positivity [hazard ratio (HR) = 2.24, 95% confidence interval (CI): 0.91–5.52; P = 0.046] is a trend of survival association in RA-ILD, but this was not confirmed in the multivariate analysis. Multivariate Cox analyses revealed that a history of smoking (HR = 2.53, 95% CI: 1.10–5.83; P = 0.030) and a systolic pulmonary artery pressure (SPAP) ≥ 37 mmHg (HR = 10.24, 95% CI: 4.07–25.77; P < 0.001) were independently associated with shorter survival in RA-ILD patients. Conclusions The prevalence of ANCAs in patients with RA-ILD is high, and ANCA testing could be considered in the diagnostic workup for RA-ILD. Oddly shaped cysts with or without a UIP pattern may be a characteristic chest imaging manifestation of ANCA-positive RA-ILD. More attention should be given to RA-ILD patients who have elevated SPAP. |
| format | Article |
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| institution | DOAJ |
| issn | 1471-2466 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | BMC |
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| series | BMC Pulmonary Medicine |
| spelling | doaj-art-3b1c0d29a0ed4e40b415f18c26c6d56c2025-08-20T03:18:34ZengBMCBMC Pulmonary Medicine1471-24662025-04-0125111010.1186/s12890-025-03644-7Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung diseaseZhe Huang0Tingting Wu1Rongdan Lu2Haijun Zhou3Yun Zhang4Li Huang5Yongxiong Gan6Hequn He7Department of Emergency, The First Affiliated Hospital of Ningbo UniversityDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo UniversityDepartment of Gastroenterology, The First Affiliated Hospital of Ningbo UniversityDepartment of Rheumatology, The First Affiliated Hospital of Ningbo UniversityDepartment of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo UniversityDepartment of Emergency, The First Affiliated Hospital of Ningbo UniversityDepartment of Emergency, The First Affiliated Hospital of Ningbo UniversityDepartment of Emergency, The First Affiliated Hospital of Ningbo UniversityAbstract Background Patients with rheumatoid arthritis (RA) or rheumatoid arthritis-associated interstitial lung disease (RA-ILD) are commonly positive for antineutrophil cytoplasmic antibodies (ANCAs). The causal relationship between RA-ILD and ANCAs and the role of ANCAs in RA-ILD remain unclear. The purpose of this study was to estimate the prevalence of ANCAs in RA-ILD patients and to investigate the clinical characteristics and outcomes of ANCA-positive RA-ILD patients. Methods Data from 104 RA-ILD patients with available ANCA results at our centre from March 2010 to June 2024 were retrospectively reviewed. ANCA positivity was defined as the presence of any one or a combination of perinuclear ANCAs (P-ANCAs), cytoplasmic ANCAs (C-ANCAs), anti-MPO or anti-PR-3. Clinical data from each patient’s initial diagnosis were collected and analysed. The clinical characteristics and survival of the ANCA-positive and ANCA-negative RA-ILD groups were compared. Results Thirty-three out of the 104 (31.7%) RA-ILD patients were positive for ANCAs. The percentages of patients positive for P-ANCAs, MPO-ANCAs, C-ANCAs, and PR3-ANCAs were 27.9% (29/104), 1.9% (2/104), 1.9% (2/104), and 1.0% (1/104), respectively. Compared with ANCA-negative RA-ILD patients, a greater proportion of ANCA-positive RA-ILD patients had respiratory symptoms, serum autoantibody (ANA) positivity, poorer baseline pulmonary function, and acute exacerbations of ILD (AE-ILD). The usual interstitial pneumonia (UIP) pattern (57.6%) was the most common chest high-resolution computed tomography (HRCT) pattern observed. ANCA-positive RA-ILD patients were more likely to have traction bronchiectasis (P = 0.029), honeycombing (P < 0.001), and oddly shaped cysts (P = 0.020) than were ANCA-negative RA-ILD patients. Univariate Cox analysis revealed that P-ANCA positivity [hazard ratio (HR) = 2.24, 95% confidence interval (CI): 0.91–5.52; P = 0.046] is a trend of survival association in RA-ILD, but this was not confirmed in the multivariate analysis. Multivariate Cox analyses revealed that a history of smoking (HR = 2.53, 95% CI: 1.10–5.83; P = 0.030) and a systolic pulmonary artery pressure (SPAP) ≥ 37 mmHg (HR = 10.24, 95% CI: 4.07–25.77; P < 0.001) were independently associated with shorter survival in RA-ILD patients. Conclusions The prevalence of ANCAs in patients with RA-ILD is high, and ANCA testing could be considered in the diagnostic workup for RA-ILD. Oddly shaped cysts with or without a UIP pattern may be a characteristic chest imaging manifestation of ANCA-positive RA-ILD. More attention should be given to RA-ILD patients who have elevated SPAP.https://doi.org/10.1186/s12890-025-03644-7Rheumatoid arthritisInterstitial lung diseaseAnti-neutrophil cytoplasmic antibodyUsual interstitial pneumonia |
| spellingShingle | Zhe Huang Tingting Wu Rongdan Lu Haijun Zhou Yun Zhang Li Huang Yongxiong Gan Hequn He Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease BMC Pulmonary Medicine Rheumatoid arthritis Interstitial lung disease Anti-neutrophil cytoplasmic antibody Usual interstitial pneumonia |
| title | Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease |
| title_full | Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease |
| title_fullStr | Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease |
| title_full_unstemmed | Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease |
| title_short | Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease |
| title_sort | prevalence and clinical significance of anti neutrophil cytoplasmic antibodies in rheumatoid arthritis associated interstitial lung disease |
| topic | Rheumatoid arthritis Interstitial lung disease Anti-neutrophil cytoplasmic antibody Usual interstitial pneumonia |
| url | https://doi.org/10.1186/s12890-025-03644-7 |
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