RT Journal Article
SR Electronic
T1 KL-6 But Not CCL-18 Is a Predictor of Early Progression in Systemic Sclerosis-related Interstitial Lung Disease
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1153
OP 1158
DO 10.3899/jrheum.170518
VO 45
IS 8
A1 Gloria A. Salazar
A1 Masataka Kuwana
A1 Minghua Wu
A1 Rosa M. Estrada-Y-Martin
A1 Jun Ying
A1 Julio Charles
A1 Maureen D. Mayes
A1 Shervin Assassi
YR 2018
UL http://www.jrheum.org/content/45/8/1153.abstract
AB Objective. The 2 pneumoproteins, KL-6 and CCL-18, are promising biomarkers in systemic sclerosis (SSc)-related interstitial lung disease (ILD). Our goal was to determine their predictive significance for forced vital capacity % (FVC%) decline within the first year of followup in patients with early SSc-ILD.Methods. Early SSc patients with imaging-verified ILD enrolled in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) cohort were included. Annualized rate of change in FVC% based on the baseline and followup measurement within 12–18 months was used as the surrogate outcomes for ILD progression.Results. Eighty-two early SSc-ILD patients with mean disease duration of 2.3 years were investigated. FVC% change ranged from −23% to 38%. Baseline KL-6 levels were higher in patients than healthy controls (p < 0.0001). Higher KL-6 levels were predictive of faster FVC% decline at the 1-year followup (r = −0.23, p = 0.037). Upon categorizing KL-6 using a previously published cutoff of 1273 U/ml, its predictive significance remained in the univariable model (b = −0.07, p = 0.01), indicating that patients with positive KL-6 had on average 7% more decline in annualized percent change of FVC%. Moreover, KL-6 remained an independent predictor after adjustment for sex, disease type, anti-Scl-70, and immunosuppressive treatment status in multivariable models. Although CCL-18 was higher in patients than controls (p < 0.001), its levels did not predict FVC decline rate (p = 0.458).Conclusion. KL-6 but not CCL-18 is predictive of early SSc-ILD progression. KL-6 is a promising pneumoprotein that can contribute to SSc-ILD clinical trial enrichment.