AI Article Synopsis
- * Researchers conducted a cross-sectional analysis involving 489 SSc-ILD patients, classifying their HRCTs to determine the presence of UIP.
- * Results showed that while MUC5B was not linked to UIP patterns, the FAM13A gene was associated with an increased likelihood of definite UIP in these patients, suggesting different genetic influences compared to idiopathic pulmonary fibrosis (IPF) and rheumatoid arthritis-related ILD.
Article Abstract
Objectives: The MUC5B promoter single nucleotide polymorphism (SNP) rs35705950 has been associated with idiopathic pulmonary fibrosis (IPF) and rheumatoid arthritis (RA)-related interstitial lung disease (ILD), but not with systemic sclerosis (SSc)-ILD. We hypothesized that the MUC5B promoter polymorphism or other IPF susceptibility loci are associated with an increased risk for the uncommon SSc-usual interstitial pneumonia (UIP) endophenotype, rather than SSc-ILD in general.
Methods: We performed a cross-sectional study of SSc-ILD patients from 4 US Scleroderma Programs to investigate the frequency of MUC5B rs35705950 and 12 additional IPF susceptibility loci. SSc-ILD patients were stratified by high resolution chest CT (HRCT) imaging findings into UIP and non-UIP groups. Analysis of HRCTs performed by a thoracic radiologist blinded to participants' characteristics classified each scan as definite UIP, probable UIP, indeterminate, or alternative diagnosis, according to American Thoracic Society criteria.
Results: Four-hundred eighty-nine SSc-ILD patients were included; 80% were female and 75% were White. Twenty-three (4.7%) patients had a definite UIP pattern. The MUC5B SNP rs35705950 was not associated with a definite UIP pattern in SSc-ILD. In contrast, patients carrying 2 copies of the IPF risk gene FAM13A minor allele rs2609255 had significantly higher odds of a definite UIP pattern compared with the other patterns (OR 3.40, 95% CI 1.19-9.70), and compared with an alternative diagnosis (OR 3.65, 95% CI 1.25-10.65).
Conclusion: We demonstrated a novel association between FAM13A and SSc-UIP. Contrary to IPF and RA-ILD, the MUC5B promoter polymorphism was not associated with a definite UIP pattern in SSc-ILD.
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| http://dx.doi.org/10.1093/rheumatology/keae573 | DOI Listing |
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Article Synopsis
- * Researchers conducted a cross-sectional analysis involving 489 SSc-ILD patients, classifying their HRCTs to determine the presence of UIP.
- * Results showed that while MUC5B was not linked to UIP patterns, the FAM13A gene was associated with an increased likelihood of definite UIP in these patients, suggesting different genetic influences compared to idiopathic pulmonary fibrosis (IPF) and rheumatoid arthritis-related ILD.
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