In:
European Journal of Clinical Investigation, Wiley, Vol. 51, No. 8 ( 2021-08)
Kurzfassung:
Krebs von den Lungen‐6 (KL‐6) is a high‐molecular‐weight (200kDa) glycoprotein proposed as a diagnostic biomarker for differentiating interstitial lung disease (ILD). Systemic sclerosis (SSc) is a rare immune‐mediated disorder, and ILD is the leading cause of morbidity and mortality. Pleuroparenchymal fibroelastosis (PPFE) has been described to have a poor prognosis in SSc‐ILD patients. This study undertook to compare serial changes in KL‐6 in SSc‐ILD patients with and without PPFE, to verify its prognostic value as a disease biomarker. Materials and Methods Twenty‐five SSc‐ILD patients (median IQR, 62 (56‐58); 20% males) were retrospectively enrolled. 12 SSc‐ILD patients (48%) had also a radiological diagnosis of PPFE. Serum KL‐6 concentrations were measured by KL‐6 reagent assay (Fujirebio Europe, Ghent, Belgium). Results Serum KL‐6 measurements were increased in SSc‐ILD patients with and without PPFE compared with healthy controls ( P 〈 .0001). Comparative analysis of the rate of variation of KL‐6 over the 6 years of follow‐up was performed by serial two‐yearly KL‐6 measurements: Δ1(t1‐t0), Δ2(t2‐t1) and Δ3(t3‐t2). In SSc‐ILD patients with PPFE pattern, Δ3 was significantly different than those without PPFE pattern ( P = .0020). Serum KL‐6 levels were significantly different ( P = .0455) either at Δ2 and Δ3 in the PPFE group. In SSc‐ILD patients with PPFE, at t3 serum KL‐6 concentrations were inversely correlated with FEV1 ( r = −.76; P = .037) and FVC percentages ( r = −.79; P = .028). Conclusion These results suggest that serial measurements of KL‐6 in the follow‐up of these patients may help to monitor disease progression. In real life, in SSc‐ILD patients PPFE should be always evaluated at CT and when present should suggest a tight follow‐up to monitor its evolution.
Materialart:
Online-Ressource
ISSN:
0014-2972
,
1365-2362
Sprache:
Englisch
Verlag:
Wiley
Publikationsdatum:
2021
ZDB Id:
2004971-7
