Table 5.

Studies published to date on KL-6 and CCL-18 in systemic sclerosis-related interstitial lung disease.

Author	Population	Outcomes Studied	Results
KL-6
Sato, et al17	45 Japanese SSc, 15 HC	Cross-sectional correlation with fibrosis scores and PFT	Inverse correlation between KL-6 and DLCO% and VC
Hant, et al18	66 SSc,10 HC	Concomitant alveolitis based on BAL or HRCT findings	KL-6 was associated with concomitant alveolitis
Hesselstrand, et al19	15 early SSc,12 HC	BAL measures, HRCT and PFT findings	KL-6 correlated with BAL eosinophil concentration.
Kuwana, et al2	50 Japanese early (< 3 yrs) SSc with ILD	ESLD (FVC < 50%, oxygen requirement	KL-6 predictive of ESLD development or death)
CCL-18
Kodera, et al24	123 SSc (86 with ILD), 37 HC	SSc to HC comparison, fibrosis score per imaging (HRCT), DLCO, VC	CCL-18 associated with decreased DLCO, VC, and higher fibrosis scores
Prasse, et al22	12 SSc-ILD, 43 IIP, 23 HC	CCL-18 levels in IIP, SSc and HC, correlation with PFT	Higher in SSc serum and BAL and inversely correlated with concomitant TLC
Tiev, et al20	83 SSc (46 with ILD)	10% decrease of baseline TLC and/or FVC% or death	CCL-18 was predictive of ILD event
Elhaj, et al21	266 early SSc (with or without ILD)	Decline in FVC%	CCL18 predicted short-term but not longterm decline in FVC
Schupp, et al23	96 SSc	> 10% FVC drop or death	Higher risk of death or lung function worsening within 2 yrs
Hoffmann-Vold, et al4	298 SSc (with or without ILD)	Rates of FVC decline and cumulative survival	Higher rate of decline in FVC and lower overall survival in patients with high CCL-18

SSc: systemic sclerosis; HC: healthy controls; ILD: interstitial lung disease; BAL: bronchoalveolar lavage; HRCT: high-resolution computed tomography; PFT: pulmonary function tests; VC: vital capacity; ESLD: endstage lung disease; IIP: idiopathic interstitial pneumonia; TLC: total lung capacity; FVC%:% forced vital capacity.