Prevalence and clinical significance of circulating autoantibodies in idiopathic pulmonary fibrosis
Open accessAbstract
Background The clinical significance of circulating autoantibodies in idiopathic pulmonary fibrosis is unclear. The objective of this study was to determine the frequency and clinical significance of circulating autoantibodies in idiopathic pulmonary fibrosis.Methods We measured an extensive panel of autoantibodies (including rheumatoid factor, anti-cyclic citrullinated peptide, and anti-nuclear antibodies by immunofluorescence) associated with connective tissue disease or vasculitis in a cohort of well-characterized patients with idiopathic pulmonary fibrosis (n = 67). The prevalence of circulating autoantibodies was compared between idiopathic pulmonary fibrosis patients and healthy controls (n = 52). We compared the clinical characteristics of patients with and without circulating autoantibodies, and analyzed the relationship between autoantibody positivity and transplant-free survival time.Results Positive autoantibodies were found in 22% of patients with IPF and 21% of healthy controls. There were no differences in the types of autoantibodies found between patients with idiopathic pulmonary fibrosis and healthy controls. Among patients with idiopathic pulmonary fibrosis, there were no significant differences in clinical characteristics between those with and without circulating autoantibodies. The presence of circulating autoantibodies was associated with longer transplant-free survival time on adjusted analysis, however the significance varied depending on which statistical model was used (HR 0.22-0.47, p value 0.02-0.17).Conclusions The frequency of circulating autoantibodies in patients with idiopathic pulmonary fibrosis is no different compared to healthy controls, but may be associated with longer survival.
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A variety of novel autoantibodies targeting neoepitopes have been reported in IPF, with many targets being structural cell proteins such as desmoplakin and periplakin.
“A variety of novel autoantibodies targeting neoepitopes have been reported in IPF (159–163), with many of the targets being structural cell proteins, such as desmoplakin (164) and periplakin (159).”
From The Role of Immune and Inflammatory Cells in Idiopathic Pulmonary Fibrosis (2018) · cites this paper as [160]
Patients with low-level titers of autoantibodies might have worsened clinical outcomes than those lacking these findings.
“patients with low level titers of autoantibodies might have worsened clinical outcomes than those patients lacking these findings (160).”
From The Role of Immune and Inflammatory Cells in Idiopathic Pulmonary Fibrosis (2018) · cites this paper as [160]
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- Authors
- Lee, J. S., Kim, E. J., Lynch, K. L., Elicker, B., Ryerson, C. J., Katsumoto, T. R., Shum, A. K., Wolters, P. J., Cerri, S., Richeldi, L., Jones, K. D., King, T. E., & Collard, H. R.
- Affiliations
- University of California, San Francisco (US)
- University of British Columbia (CA)
- University of Modena and Reggio Emilia (IT)
- Corresponding author
- Lee, Joyce S.
- ORCID
- Venue
- Respiratory Medicine
- Publisher
- Elsevier BV
- Published
- 2013-02
- Volume
- 107
- Issue
- 2
- Pages
- 249-255
- ISSN
- 0954-6111 (ISSN-L)
- PMC
- PMC3543473
- OpenAlex
- W2160384627
- Cited by
- 100
- References
- 27
- Citations by year
- 2026: 2 · 2025: 4 · 2024: 4 · 2023: 10 · 2022: 4 · 2021: 9 · 2020: 6 · 2019: 12 · 2018: 14 · 2017: 7 · 2016: 6 · 2015: 8 · 2014: 9 · 2013: 5
- FWCI
- 5.68
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Respiratory Medicine (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- IRIS UNIMORE (University of Modena and Reggio Emilia) (submittedVersion, cc-by)
- ePrints Soton (University of Southampton) (submittedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Funders
- National Institutes of Health
- University of California, San Francisco
- National Heart, Lung, and Blood Institute (HL086516, F32HL097383, HL097383, K08HL095659, K23HL086516)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
- Keywords
- idiopathic pulmonary fibrosis, serum autoantibodies, autoantibody prevalence, rheumatoid factor, anti-CCP antibodies, antinuclear antibodies, connective tissue diseases, vasculitis, transplant-free survival, prognosis
- MeSH terms
- Aged, Aged, 80 and over, Autoantibodies, Female, Humans, Male, Middle Aged, Phenotype, Prognosis, Case-Control Studies, Lung Transplantation, Kaplan-Meier Estimate, Idiopathic Pulmonary Fibrosis
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