Spliceosome-associated protein 130: a novel biomarker for idiopathic pulmonary fibrosis.

BACKGROUND

Spliceosome-associated protein 130 (SAP130), a novel danger-associated molecular pattern (DAMP), is involved in inflammatory disease. However, no data are available about SAP130 in idiopathic pulmonary fibrosis (IPF). Our study aimed to investigate SAP130 in the serum and lung tissue of patients with IPF and to determine its clinical significance.

METHODS

SAP130 levels in the serum of 83 IPF patients and 38 healthy subjects were measured. Additionally, immunohistochemical staining for SAP130 was performed in lung specimens of IPF patients and control subjects. Correlation between serum SAP130 levels and clinical parameters were investigated.

RESULTS

Serum SAP130 levels were elevated in IPF patients compared with healthy controls. In parallel, the expression of SAP130 in lung tissue was elevated in IPF. SAP130 levels were higher in patients with acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) than patients with stable IPF (P=0.0144). The area under curve (AUC) of the ROC curve for the diagnosis of IPF was 0.944 (95% CI, 0.810-0.997) for SAP130. The sensitivity (92.1%) and specificity (69.9%) were obtained for the cutoff value of 643.87 pg/mL. In patients with stable IPF, the SAP130 level correlated positively with fibrosis on high-resolution CT (HRCT) (r=0.4164, P=0.0029) and serum KL-6 (r=0.4564, P=0.0010), and inversely with FEV1 (r=-0.3562, P=0.0120) and DLCO (r=-0.5550, P<0.0001). In patients with AE-IPF, the SAP130 level correlated positively with fibrosis (r=0.3735, P=0.0296) and ground-glass opacity (r=0.4697, P=0.0051) on HRCT and serum Krebs von den Lungen 6 (KL-6) (r=0.5470, P= 0.0008).

CONCLUSIONS

The study suggested that SAP130 was a potential noninvasive biomarker that correlates well with disease severity of IPF. A prospective, multicentre study is required to validate the clinical and pathophysiological utility of SAP130 in IPF.