Table 2.

Novel blood-based biomarkers in patients with systemic sclerosis associated interstitial lung disease

Biomarker	Abbreviation	Increased expression / association / correlation	Reference(s)
Clara Cell 16-kDa	CC16	Active ILD	(1)
Chemokine (C-C motif) ligand 2	CCL2	Skin and lung disease activity	(2)
Chemokine (C-C motif) ligand 18	CCL18	Fibrotic activity in the lungs	(3, 4)
Connective Tissue Growth Factor	CTGF	Extent of skin and lung fibrosis	(5)
Chemokine (C-X3-C motif) ligand 1	CX3CL1	Progressive ILD	(6)
Chemokine (C-X-C motif) ligand 10	CXCL10	Active / progressive ILD	(7, 8)
Chemokine (C-X-C motif) ligand 16	CXCL16	Active ILD	(9)
Chemokine (C-X-C motif) ligand 4	CXCL4	Progressive ILD	(10)
Chemokine (C-X-C motif) ligand 9	CXCL9	ILD	(11)
E-Selectin	E-Selectin	Progressive ILD	(12–14)
Endothelin-1	ET-1	ILD	(14)
Growth Differentiation Factor-15	GDF-15	Extent of skin and lung fibrosis	(15–18)
Gremlin-1	Gremlin-1	ILD in early diffuse systemic sclerosis	(19)
Human epididymis protein 4	HE-4	ILD	(20)
Intercellular Adhesion Molecule 1	ICAM-1	ILD	(21)
Interleukin-4	IL-4	ILD	(22)
Interleukin −6	IL-6	ILD	(23)
Interleukin-22	IL-22	ILD	(23)
Interleukin-23	IL-23	ILD	(24)
Interleukin-33	IL-33	Extent of skin and lung fibrosis	(18)
Interleukin-35	IL-35	Severity of lung fibrosis	(25)
Krebs von den Lungen-6	KL-6	Active ILD and severity of fibrosis	(1, 3, 26–28)
Matrix Metalloproteinase-12	MMP-12	Severity of skin and lung fibrosis	(29)
Matrix Metalloproteinase-7	MMP-7	Poor baseline lung function and transplant-free survival	(30)
Pulmonary and activation-regulated chemokine	PARC	Fibrotic activity in the lungs	(28)
Surfactant Protein-A	SP-A	ILD	(31)
Surfactant Protein-D	SP-D	Active ILD	(1, 26, 27, 32–34)
Tissue inhibitors of metalloproteinase 1	TIMP-1	ILD	(35)
Vascular Endothelial Growth Factor	VEGF	ILD	(14)
YKL-40	YKL-40	ILD	(36)