S143 Serum CYFRA 21–1 as a prognostic marker in scleroderma-associated interstitial lung disease

Abstract

Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc). The progression of SSc associated ILD (SSc-ILD) is highly variable. Markers predictive of progressive ILD are needed early in the disease course, to appropriately target patients at risk of developing progressive pulmonary fibrosis. CYFRA 21–1 (CYFRA) is a tumour marker expressed by type I/type II pneumocytes and respiratory bronchiolar epithelial cells, released into the blood following cell lysis. Serum levels of CYFRA were measured in both a retrospective (n=180) and a prospective (n=118) cohort of SSc patients. Retrospective cohort mean age: 49.1 (range 47.08–51.05), 77.25% female, prospective cohort age: 56.4 (54.10–58.73), 76.23% female. Median FVC% predicted and DLCO% predicted: retrospective cohort: 80.1% (IQR:67.2–95.5) and 55.5% (44.3–68.35), respectively – prospective cohort: 73.8% (57.2–87) and 39.9% (29.2–48.8), respectively. ILD was defined as the presence of fibrosis on chest imaging (chest x-ray or HRCT) and/or a forced vital capacity (FVC)