Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis
Abstract Background Patients with interstitial lung disease (ILD) who show features related to autoimmunity without meeting criteria for a defined connective tissue disease are categorized as interstitial pneumonia with autoimmune features (IPAF). The present study compared clinical characteristics...
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| Format: | Article |
| Language: | English |
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BMC
2019-07-01
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| Series: | BMC Pulmonary Medicine |
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| Online Access: | http://link.springer.com/article/10.1186/s12890-019-0868-9 |
| _version_ | 1819008499850412032 |
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| author | Jeong Uk Lim Bo Mi Gil Hye Seon Kang Jongyeol Oh Yong Hyun Kim Soon Seog Kwon |
| author_facet | Jeong Uk Lim Bo Mi Gil Hye Seon Kang Jongyeol Oh Yong Hyun Kim Soon Seog Kwon |
| author_sort | Jeong Uk Lim |
| collection | DOAJ |
| description | Abstract Background Patients with interstitial lung disease (ILD) who show features related to autoimmunity without meeting criteria for a defined connective tissue disease are categorized as interstitial pneumonia with autoimmune features (IPAF). The present study compared clinical characteristics and clinical outcomes of patients with IPAF to patients with connective tissue disease related-interstitial lung disease (CTD-ILD) and patients with idiopathic pulmonary fibrosis (IPF). Methods ILD patients who were consecutively enrolled in a single institution ILD cohort between 2008 and 2015 were evaluated for the study. Clinical data had been prospectively collected, while radiologic imaging and pathologic findings were re-reviewed for the present study. Results Out of 305 patients with ILD, 54 (17.7%) patients met the classification of IPAF, 175 (57.4%) patients had IPF, and 76 (24.9%) patients were diagnosed with CTD-ILD. Compared to IPF, incidences of acute exacerbations in 1,3 and 5 years were significantly less in the IPAF group (p = 0.022, p = 0.026 and p = 0.007, respectively). From multivariate analysis for mortality, age (p = 0.034, HR 1.022, 95% CI: 1.002–1.044), FVC (p < 0.001, HR 0.970, 95% CI: 0.955–0.984), ILD exacerbation (p = 0.001, HR 2.074, 95% CI: 1.366–3.148), and ILD type (p = 0.047, HR 0.436, 95% CI: 0.192–0.984 (IPAF vs IPF), respectively) showed significant association. Conclusions Compared to the other ILD groups, IPAF showed distinct clinical characteristics. The IPAF group showed better survival and less episodes of exacerbation when compared to the IPF group. |
| first_indexed | 2024-12-21T00:41:27Z |
| format | Article |
| id | doaj.art-aa2878105b854e8e82cf99537eff3767 |
| institution | Directory Open Access Journal |
| issn | 1471-2466 |
| language | English |
| last_indexed | 2024-12-21T00:41:27Z |
| publishDate | 2019-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pulmonary Medicine |
| spelling | doaj.art-aa2878105b854e8e82cf99537eff37672022-12-21T19:21:41ZengBMCBMC Pulmonary Medicine1471-24662019-07-0119111010.1186/s12890-019-0868-9Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosisJeong Uk Lim0Bo Mi Gil1Hye Seon Kang2Jongyeol Oh3Yong Hyun Kim4Soon Seog Kwon5Division of Pulmonary and Critical Care Medicine, Department of Medicine, The Armed Forces Capital HospitalDepartment of Radiology, Bucheon St Mary’s Hospital, College of Medicine, The Catholic University of KoreaDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of KoreaDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of KoreaDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of KoreaDivision of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of KoreaAbstract Background Patients with interstitial lung disease (ILD) who show features related to autoimmunity without meeting criteria for a defined connective tissue disease are categorized as interstitial pneumonia with autoimmune features (IPAF). The present study compared clinical characteristics and clinical outcomes of patients with IPAF to patients with connective tissue disease related-interstitial lung disease (CTD-ILD) and patients with idiopathic pulmonary fibrosis (IPF). Methods ILD patients who were consecutively enrolled in a single institution ILD cohort between 2008 and 2015 were evaluated for the study. Clinical data had been prospectively collected, while radiologic imaging and pathologic findings were re-reviewed for the present study. Results Out of 305 patients with ILD, 54 (17.7%) patients met the classification of IPAF, 175 (57.4%) patients had IPF, and 76 (24.9%) patients were diagnosed with CTD-ILD. Compared to IPF, incidences of acute exacerbations in 1,3 and 5 years were significantly less in the IPAF group (p = 0.022, p = 0.026 and p = 0.007, respectively). From multivariate analysis for mortality, age (p = 0.034, HR 1.022, 95% CI: 1.002–1.044), FVC (p < 0.001, HR 0.970, 95% CI: 0.955–0.984), ILD exacerbation (p = 0.001, HR 2.074, 95% CI: 1.366–3.148), and ILD type (p = 0.047, HR 0.436, 95% CI: 0.192–0.984 (IPAF vs IPF), respectively) showed significant association. Conclusions Compared to the other ILD groups, IPAF showed distinct clinical characteristics. The IPAF group showed better survival and less episodes of exacerbation when compared to the IPF group.http://link.springer.com/article/10.1186/s12890-019-0868-9Interstitial pneumonia with autoimmune featuresInterstitial lung diseaseConnective tissue diseaseIdiopathic pulmonary fibrosis |
| spellingShingle | Jeong Uk Lim Bo Mi Gil Hye Seon Kang Jongyeol Oh Yong Hyun Kim Soon Seog Kwon Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis BMC Pulmonary Medicine Interstitial pneumonia with autoimmune features Interstitial lung disease Connective tissue disease Idiopathic pulmonary fibrosis |
| title | Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis |
| title_full | Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis |
| title_fullStr | Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis |
| title_full_unstemmed | Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis |
| title_short | Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis |
| title_sort | interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis |
| topic | Interstitial pneumonia with autoimmune features Interstitial lung disease Connective tissue disease Idiopathic pulmonary fibrosis |
| url | http://link.springer.com/article/10.1186/s12890-019-0868-9 |
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