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Updated: Jul 19, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Increased levels of KL-6 and subsequent mortality in patients with interstitial lung diseases
H Satoh1, K Kurishima, H Ishikawa
1Division of Respiratory Medicine, Institute of Clinical Medicine, University of Tsukuba, Tsukuba City, Ibaraki, Japan. hirosato@md.tsukuba.ac.jp
Insights
Elevated KL-6 levels in interstitial lung disease (ILD) patients indicate a higher risk of mortality. A cut-off of 1000 U/mL effectively identifies patients with a poor prognosis, aiding in risk stratification.
Area of Science:
- Pulmonary Medicine
- Biomarkers
- Prognostic Indicators
Background:
- Interstitial lung diseases (ILDs) are a group of serious respiratory conditions.
- KL-6 is a known biomarker for ILDs, but its prognostic value for mortality is not fully established.
- Accurate prognostic markers are crucial for managing ILD patients.
Purpose of the Study:
- To investigate the association between elevated serum KL-6 levels and mortality in patients with stable-state ILDs.
- To determine a reliable cut-off level for KL-6 to differentiate between good and poor prognosis in ILD patients.
Main Methods:
- A cohort of 219 ILD patients (idiopathic interstitial pneumonia and collagen disease-associated pulmonary fibrosis) were studied.
- Serum KL-6 levels were measured using an enzyme immunoassay kit.
- Patients were followed up to assess mortality, with statistical analyses including ROC curve, univariate, and multivariate Cox proportional hazard models.
Main Results:
- Patients who died from respiratory failure had significantly higher serum KL-6 levels (P = 0.0004).
- Receiver operating characteristic (ROC) curve analysis identified 1000 U/mL as the optimal cut-off for distinguishing prognosis.
- Elevated KL-6 (>1000 U/mL) was a significant independent predictor of poor prognosis in both univariate and multivariate analyses (P < 0.001).
Conclusions:
- Elevated serum KL-6 levels serve as a simple yet valuable prognostic marker for identifying ILD patients at increased risk of mortality.
- The established cut-off of 1000 U/mL can aid clinicians in risk stratification and patient management.
- Further research may explore the clinical utility of KL-6 in guiding treatment decisions for ILDs.
Objectives:
KL-6 is a specific marker in patients with interstitial lung diseases (ILDs); however, the relationship between elevated levels of KL-6 and subsequent mortality is not well defined. To determine if elevated serum levels of KL-6 are associated with increased mortality, and to identify the most suitable cut-off level of KL-6 by which to distinguish between good prognosis and poor prognosis, we evaluated the prognostic significance of serum KL-6 levels in patients with stable-state ILDs.
Methods:
Two hundred and nineteen patients diagnosed with ILDs (152 with idiopathic interstitial pneumonia and 67 with collagen disease-associated pulmonary fibrosis) at Tsukuba University Hospital from April 1999 to October 2005 were entered in this study. Serum KL-6 levels in patients with ILDs were measured with a commercially available enzyme immunoassay kit, and these patients were then followed up.
Results:
During the follow-up period, 58 of the 219 patients died of respiratory failure. Patients who died during this period had higher levels of KL-6 than did those who did not (P = 0.0004). The receiver operating characteristic curve analysis showed 1000 U mL(-1) as the most suitable cut-off level by which to distinguish between the two groups of patients. The 95% specificity serum KL-6 level with poor outcome was 2750 U mL(-1). In univariate and multivariate analysis, elevated serum KL-6 (>1000 U mL(-1)) in the stable state indicated poor prognosis (P = 0.0005, log-rank test; P = 0.0001, Cox proportional hazard model).
Conclusions:
Elevated KL-6 level may provide simple, yet valuable information by which to identify patients with ILDs who are at increased risk for subsequent mortality.
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