Clinical Features Of Connective Tissue Disease-associated Interstitial Lung Disease Complicated With Pulmonary Hypertension

Objective:To analyze the clinical features of connective tissue disease-associated interstitial lung disease complicated with pulmonary hypertension（CTD-ILD-PH）to improve the understanding of this group of patients.Methods:180 patients with connective tissue disease-associated interstitial lung disease who were treated in the First Hospital of Lanzhou University from January2018 to August 2021 were enrolled to our study.According to the pulmonary arterial systolic pressure（PASP）measured by echocardiography,the patients were divided into pulmonary hypertension group（PH group）and non-pulmonary hypertension group（non-PH group）.Compared the differences of general condition,clinical manifestations,serological indexes,Imaging features between the two groups,and analyzed the clinical features of CTD-ILD-PH.Results:（1）General condition:Among 180 cases of CTD-ILD,85 cases（47.2%）were in the PH group and 95 cases（52.8%）in the non-PH group.Both groups were mostly female,61 cases and 68 cases,respectively.The mean age was 64.17±13.17 years and 59.41±13.69 years,respectively.The Body Mass Index（BMI）was22.61±3.61kg/m~2and 22.03±3.53 kg/m~2,respectively.There was no significant difference in gender and BMI between the two groups（P>0.05）,but there was significant difference in age（P<0.05）.（2）Clinical manifestations:Among the 180 patients included,the clinical manifestations were chest tightness and shortness of breath in 119 cases,cough and sputum in 117 cases,arthralgia/limb swelling in 92 cases,Velcro crackle in 56 cases,dry mouth/eyes in 48 cases,morning stiffness in 41 cases,fever in 33 cases,Raynor’s phenomenon in 25 cases,rash in 22 cases,limb numbness/weakness in 16 cases,and acropachy in 3 cases.Analysis and comparison of the two groups showed:chest tightness and shortness of breath,cough and sputum,Raynor’s phenomenon were more common in the PH group（P<0.05）,and there were no significant differences in other clinical manifestations between the two groups.（3）Serological indexes:NLR,RDW,MPV,CRP,TBIL,LDH,Crea,UA,HCY,D-D and BNP in PH group were higher than those in non-PH group（P<0.05）.The positive rates of ANA,AMA-M2,Ro-52,SSA,anti-n RNP and anti-CEMP antibodies were higher in PH group（P<0.05）.Correlation analysis showed that NLR,RDW,MPV,CRP,LDH,UA,HCY,D-D,BNP and PASP levels were positively correlated.ANA,AMA-M2,Ro-52,SSA,anti-n RNP and anti-CEMP antibodies were positively correlated with pulmonary hypertension.Regression analysis found that age,increased RDW,increased MPV,and increased BNP were the risk factors for CTD-ILD with PH.The results of drawing the ROC curve showed that the AUC of BNP in the diagnosis of PH was 0.770,the sensitivity was 68.9%,and the specificity was 85.7%;the AUC of HCY in the diagnosis of PH was 0.662,the sensitivity was57.6%,and the specificity was 71.6%;the AUC of MPV in the diagnosis of PH was0.661.The sensitivity was 87.1%,and the specificity was 48.4%.（4）HRCT examination:Among the 180 patients included,grid shadows（106 cases）were the most common findings on chest HRCT,followed by linear opacities（104cases）,pleural thickening（103 cases）,nodular shadows（94 cases）,mediastinal lymphadenopathy（88 cases）,ground glass opacities（85 cases）,patch shadow（66cases）,cystic shadow（44 cases）,honeycomb shadow（44 cases）,and interlobular septal thickening（35 cases）.Grid shadow,ground glass opacities,interlobular septal thickening and mediastinal lymphadenopathy were more common in the PH group（P<0.05）,while other imaging manifestations showed no significant differences between the two groups.Results:1.Patients with CTD-ILD complicated with pulmonary hypertension had more obvious chest tightness and shortness of breath,cough and sputum,Raynaud’s phenomenon;2.NLR,RDW,MPV,CRP,LDH,UA,HCY,D-D,TBIL,BNP were positively correlated with pulmonary artery pressure,indicating that the higher the level of these above indexes,the greater the pulmonary artery pressure;RDW,MPV,and BNP can be used as risk factors for pulmonary hypertension.3.CTD-ILD high-resolution CT shows grid shadows,ground glass opacities,interlobular septal thickening,and mediastinal lymphadenopathy,which were suggestive of pulmonary hypertension.4.Autoantibodies of CTD-ILD patients with high levels of ANA,AMA-M2,Ro-52,SSA,anti-n RNP and anti-CEMP antibodies are more likely to be complicated with pulmonary hypertension,but their levels are not related to the severity of pulmonary hypertension.