Journal article: Choline and CCL22 Are Prognostic Blood Biomarkers for Hermansky-Pudlak Syndrome Pulmonary Fibrosis
Link: https://academic.oup.com/ajrcmb/article-abstract/74/1/80/8438562
One-Sentence Takeaway: This study found that two substances in the blood—CCL22 and choline—may help predict and track lung fibrosis in people with HPS-1, and it identified iNOS as a potential target for future treatments aimed at preventing lung scarring.
Summary: Researchers are looking for blood markers (biomarkers) that can help doctors predict and track lung scarring, also known as pulmonary fibrosis (PF). Because the disease progression is predictable, HPS-1 provides a useful model for studying PF.
The researchers analyzed blood samples from people with:
- HPS-1 without lung disease
- HPS-1 with pulmonary fibrosis (HPSPF)
- Other forms of HPS
- Idiopathic Pulmonary Fibrosis (a more common type of PF)
- Healthy volunteers
Using advanced laboratory techniques, they examined hundreds of molecules in the blood and compared the results with lung function tests and patient age.
What They Found
As pulmonary fibrosis developed and worsened in HPS-1 patients, several important changes occurred in the body’s metabolism and inflammatory pathways.
Two blood markers stood out:
- CCL22, an immune system signaling protein
- Choline, a nutrient involved in cell function and metabolism
Both markers were:
- Higher in patients who had pulmonary fibrosis
- Associated with worsening lung function
- Able to accurately predict which HPS-1 patients had developed pulmonary fibrosis
This suggests that measuring CCL22 and choline in blood could help doctors identify patients at risk for lung fibrosis and monitor disease progression.
Additional Findings
The researchers also found that HPS-related pulmonary fibrosis shares some biological features with progressive pulmonary fibrosis, suggesting that the diseases may involve similar mechanisms.
They identified an enzyme called inducible nitric oxide synthase (iNOS) that appears to trigger the release of several molecules that promote fibrosis and inflammation, including:
- CCL22
- CCL24
- IL-18
- IL-1α
- IL-1β
Why This Matters
The study suggests that:
- CCL22 and choline may be useful blood tests for predicting and monitoring lung fibrosis in people with HPS-1.
- HPS-related pulmonary fibrosis and progressive pulmonary fibrosis may develop through similar biological pathways.
- Blocking iNOS could be a promising new treatment strategy to slow or prevent lung scarring in HPS patients.