Tuesday, December 2 | 8:40 a.m.-8:50 a.m. | T1-SSCH05-5 | Room S501
In this Tuesday session, researchers will share results from a study that found fibrotic interstitial lung disease (ILD) in patients with sickle cell disease is a key indicator of future disease progression and worsening lung function.
“[Our] study demonstrates that fibrotic interstitial lung disease in patients with sickle cell disease is common and identifies baseline CT fibrotic findings as predictive of long-term ILD progression — which was associated with reduced lung function,” presenter Victoria Shi of Johns Hopkins Medicine in Baltimore and colleagues reported.
Shi’s group conducted a study that investigated the frequency of fibrotic ILD and predictors of its progression in sickle cell disease patients. The research included sickle cell patients who had undergone two chest CT exams — with the baseline and most recent scans performed at a minimum interval of five years — between 2002 and 2024.
The investigators reported the following:
- Of 133 patients diagnosed with sickle cell disease, 75.9% had the hemoglobin SS genotype, 11.3% had the hemoglobin SC genotype, and 12.8% had other genotypes.
- Fibrotic changes on CT at baseline and follow-up imaging were 33.1% and 37.6%, respectively, with a median time to follow-up of 9.7 years.
- Fibrotic interstitial lung disease progression on CT occurred at an overall rate of 12% over the follow-up period.
- Baseline fibrotic changes on CT were an independent risk factor for interstitial lung disease progression (hazard ratio, 6.9; p = 0.03).
The research results “underscore that early radiologic evaluation may help identify those who will benefit from targeted management strategies to reduce the risk of interstitial lung disease progression,” the group concluded.