Abstract
Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by dysregulation of the immune system, vascular damage, and fibrosis of the skin and internal organs. Patients with SSc show a heterogeneous phenotype and a range of clinical courses. Therefore, biomarkers that are helpful for precise diagnosis, prediction of clinical course, and evaluation of the therapeutic responsiveness of disease are required in clinical practice. SSc-specific autoantibodies are currently used for diagnosis and prediction of clinical features, as other biomarkers have not yet been fully vetted. Krebs von den Lungen-6 (KL-6), surfactant protein-D (SP-D), and CCL18 have been considered as serum biomarkers of SSc-related interstitial lung disease. Moreover, levels of circulating brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) can provide diagnostic information and indicate the severity of pulmonary arterial hypertension. Assessment of several serum/plasma cytokines, chemokines, growth factors, adhesion molecules, and other molecules may also reflect the activity or progression of fibrosis and vascular involvement in affected organs. Recently, microRNAs have also been implicated as possible circulating indicators of SSc. In this review, we focus on several potential SSc biomarkers and discuss their clinical utility.
Highlights
Systemic sclerosis (SSc) is a multifaceted chronic autoimmune disease with characteristic vascular damage and fibrosis of the skin and internal organs [1,2]
↑, upregulated; ↓, downregulated; Transforming Growth Factor (TGF)-β, transforming growth factor; Growth Differentiation Factor 15 (GDF-15), growth differentiation factor 15; BAFF, B-cell-activating factor belonging to the tumor necrosis factor family; a Proliferation-Inducing Ligand (APRIL), a proliferation-inducing ligand; Matrix Metalloproteinases (MMPs), matrix metalloproteinases; BNP, brain natriuretic peptide; NT-proBNP, N-terminal-pro hormone BNP; Connective Tissue Growth Factor (CTGF), connective tissue growth factor; mRSS, modified Rodnan total skin thickness score; ILD, interstitial lung disease; IL-6, interleukin 6; DLco, diffusing capacity of carbon monoxide; CT, computed tomography; PAH, pulmonary arterial hypertension; ICAM-1, intercellular adhesion molecule 1; dcSSc, diffuse cutaneous systemic sclerosis; VEGF, vascular endothelial growth factor; lcSSc, limited cutaneous systemic sclerosis; Krebs von den Lungen-6 (KL-6), krebs von den Lungen-6; HRCT, high resolution CT; SP-D, surfactant protein-D
The BAFF and APRIL cytokines are produced by various cells including monocytes and dendritic cells and both bind to each receptor expressed on B cells, known as B cell maturation protein (BCMA) and transmembrane activator and CAML interactor (TACI)
Summary
Systemic sclerosis (SSc) is a multifaceted chronic autoimmune disease with characteristic vascular damage and fibrosis of the skin and internal organs [1,2]. Vascular involvement, including digital ulcers and pulmonary arterial hypertension (PAH), can be detected in both lcSSc and dcSSc. The clinical course of skin sclerosis and organ lesions varies in each case. ↑, upregulated; ↓, downregulated; TGF-β, transforming growth factor; GDF-15, growth differentiation factor 15; BAFF, B-cell-activating factor belonging to the tumor necrosis factor family; APRIL, a proliferation-inducing ligand; MMP, matrix metalloproteinases; BNP, brain natriuretic peptide; NT-proBNP, N-terminal-pro hormone BNP; CTGF, connective tissue growth factor; mRSS, modified Rodnan total skin thickness score; ILD, interstitial lung disease; IL-6, interleukin 6; DLco, diffusing capacity of carbon monoxide; CT, computed tomography; PAH, pulmonary arterial hypertension; ICAM-1, intercellular adhesion molecule 1; dcSSc, diffuse cutaneous systemic sclerosis; VEGF, vascular endothelial growth factor; lcSSc, limited cutaneous systemic sclerosis; KL-6, krebs von den Lungen-6; HRCT, high resolution CT; SP-D, surfactant protein-D
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