Abstract

The course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) varies among individuals. Red blood cell distribution width (RDW) has been reported to be a predictor of idiopathic pulmonary fibrosis. However, there are no studies on the relationship between RDW and SSc-ILD. We conducted a retrospective study of 28 patients who were diagnosed with SSc-ILD on their first visit to our hospital and were followed-up for 5 years. The correlation between the changes in RDW, KL-6, and SP-D (ΔRDW, ΔKL-6, ΔSP-D) and the changes in percent-predicted forced lung volume and % carbon monoxide diffusion (Δ%FVC, Δ%DLco) was investigated. ΔRDW at 1 year after diagnosis was significantly inversely correlated with Δ%FVC at 5 years after diagnosis (r = −0.51, p < 0.001) and Δ%DLco at 5 years after diagnosis (r = −0.47, p < 0.001), whereas ΔKL-6 and ΔSP-D at 1 year were not correlated with Δ%FVC or Δ%DLco at 5 years. In the group of SSc-ILD patients with RDW increase in the first year after diagnosis, %FVC and %DLco were significantly lower than baseline at 3-, 4-, and 5-year assessments. In the group of patients without RDW increase in the first year, %FVC and %DLco did not decrease during the follow-up period. In conclusion, the changes in RDW in the first year after diagnosis may be useful surrogate markers to predict the long-term course of SSc-ILD.

Highlights

  • Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular damage and fibrosis in various organs such as skin and lungs [1,2,3]

  • We examined the correlation between the changes in Red blood cell distribution width (RDW), KL-6 (∆KL-6), and surfactant protein-D (SP-D) (∆SP-D) in 1 year after first admission and those in %FVC (∆FVC) and %DLco (∆DLco) in 5 years

  • By this retrospective study, we demonstrated that the increase in RDW, a quantitative measure of variability in the size of circulating erythrocytes, at early stage of SSc-associated interstitial lung disease (SSc-ILD) may predict the long-term deterioration of %FVC and %DLco

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Summary

Introduction

Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular damage and fibrosis in various organs such as skin and lungs [1,2,3]. SSc has a poor prognosis, especially when complicated by SSc-associated interstitial lung disease (SSc-ILD) [4,5,6]. The progression of SSc-ILD varies among individuals, making it difficult to determine which patients should be treated [3,7,8,9]. It would be desirable to identify simple prognostic factors in order to make better decisions about whether early treatment should be initiated in individual SSc-ILD patients to prevent future lung function deterioration. Among the biomarkers of SSc-ILD that can be measured by blood sampling, Krebs von den Lungen (KL-6) and surfactant protein-D (SP-D) are well known. The usefulness of both biomarkers as prognostic factors has not been clarified so far, and further studies are needed [10]

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