A Retrospective Study Evaluated The Clinical Value Of Tumor Markers And Pulmonary Arterial Pressure In Patients With Primary Sj（?）gren’s Syndrome-related Interstitial Lung Disease

Objective To investigate the clinical value of tumor markers and pulmonary artery pressure in patients with primary Sj（?）gren’s syndrome–related interstitial lung disease（pSS-ILD）with different pulmonary fibrosis patterns and different degrees of pulmonary fibrosis using high-resolution computed tomography（HRCT）and transthoracic echocardiography（TTE）.Methods This study included 208 patients with pSS with and without ILD（pSS-ILD and pSS-N-ILD）.Evaluations of HRCT patterns were based on the definition of idiopathic pulmonary fibrosis（IPF）.The degree of pulmonary fibrosis in patients with pSS-ILD was scored for the entire lung using three categories:（i）Mild fibrosis=affecting0-25% of lung parenchyma;（ii）Moderate fibrosis=affecting 26–50% of lung parenchyma;（iii）Severe fibrosis=affecting 50–100% of lung parenchyma.Levels of tumor markers including cytokeratin 19 fragments（CYFRA21-1）,carbohydrate antigen（CA）153,carcinoembryonic antigen（CEA）,CA125,CA19-9,neuron-specific enolase,alpha-fetoprotein,CA724,and serum squamous cell antigen（SCC）were obtained for all.All patients had pulmonary arterial pressure test results using TTE.According to pulmonary artery systolic pressure（PASP）results,pSS patients were divided into four groups:（i）normal pulmonary arterial pressure group（PASP<35mm Hg）,;（ii）mild pulmonary arterial hypertension（PAH）group（35≤PASP<50mm Hg）;（iii）moderate PAH group（50≤PASP<70mm Hg）;（iiii）severe PAH group（PASP≥70mm Hg）.Results Factors associated with pSS-ILD were older age,male sex,high levels of White blood cell（WBC）,neutrophil,lactate dehydrogenase（LDH）,erythrocyte sedimentation rate（ESR）and C-reactive protein（CRP）.pericardial effusion were more frequent in pSS-ILD patients when compared to pSS without ILD patients.Tumor marker levels were significantly higher in pSS-ILD patients than in those without ILD.CYFRA21-1 had the highest efficacy for the diagnosis of ILD in pSS patients.As compared with nonspecific interstitial pneumonitis patients,usual interstitial pneumonia patients had higher levels of CEA,CA153,CA125,and CA19-9.There was a moderate correlation between the degree of pulmonary fibrosis and CYFRA21-1,CA153,and CEA.In 208 patients with pSS,the overall prevalence of PAH was 36%.There was a positive correlation between the the pulmonary artery pressure and age.The Pulmonary artery pressure of pSS-ILD group was significant higher than the pSS without ILD.pulmonary artery pressure has good efficacy for the diagnosis of ILD in pSS patients。There was a Moderate correlation between the degree of pulmonary fibrosis and pulmonary artery pressure.Compared with NSIP and OP,UIP patients had higher levels of pulmonary artery pressure.Conclusions Older age,male sex,respiratory symptoms,and high levels of WBC,Neutrophils,LDH,ESR,CRP were risk factors associated with pSS-ILD.There is a positive correlation between serum tumor marker levels and pSS-ILD.Higher levels of CYFRA21-1 and CA153 suggest an increased risk of ILD and may be useful as biomarkers for detecting pSS-ILD.Elevated levels of CA125,CEA,CA153,and CA19-9 may indicate a more severe pattern of pulmonary fibrosis（UIP）.Levels of CYFRA21-1,CA153,and CEA were correlated with the degree of pulmonary fibrosis in pSS-ILD patients.Pulmonary hypertension was positively correlated with age and the degree of pulmonary fibrosis in pSS-ILD patients.Elevated pulmonary arterial pressure suggests an increased risk of ILD.The pulmonary arterial pressure in the UIP group was significantly higher than that in other ILD patterns.