Elevated serum krebs von den lungen-6 in early disease predicts subsequent deterioration of pulmonary function in patients with systemic sclerosis and interstitial lung disease

Masataka Kuwana, Yuichiro Shirai, Tsutomu Takeuchi

Research output: Contribution to journalArticle

20 Citations (Scopus)


Objective. To identify predictors of poor prognosis in patients with systemic sclerosis (SSc) associated with interstitial lung disease (ILD). Methods. Fifty patients with early-stage SSc-ILD who had never received disease-modifying drugs and were either observed for ≥ 10 years or died from ILD-related causes were enrolled. The baseline variables of patients who developed endstage lung disease (ESLD) were compared with those of patients who remained ESLD-free, and the Cox proportional hazard model was used to identify initial factors that correlated with ESLD development. Results. Sixteen patients (32%) developed ESLD during 173.5 64.7 months of followup. Elevated serum Krebs von den Lungen-6 (KL-6) at initial assessment was highly correlated with ESLD development (p = 0.0002). Receiver-operating characteristic curve analysis revealed that a KL-6 value of 1273 U/ml effectively discriminated patients who developed ESLD from those who did not. Patients with KL-6 > 1273 U/ml were less likely to remain ESLD-free compared with those with lower KL-6 levels (p < 0.0001). Multivariate analysis showed that KL-6 > 1273 U/ml was the most reliable predictor of ESLD development (OR 51.2, 95% CI 7.6-343, p < 0.0001). Finally, the initial KL-6 level correlated with the forced vital capacity (FVC) decline rate (r = 0.58, p < 0.0001). Conclusion. The natural course of SSc-ILD is highly variable. Baseline serum KL-6 is a biomarker potentially useful for predicting FVC decline.

Original languageEnglish
Pages (from-to)1825-1831
Number of pages7
JournalJournal of Rheumatology
Issue number10
Publication statusPublished - 2016 Oct 1
Externally publishedYes



  • Interstitial Lung Disease
  • Outcome Assessment
  • Survival Analysis
  • Systemic Sclerosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology

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