| Background&Objective:Connective tissue disease（CTD）can involve the interstitium,airways,pleura and pulmonary vasculature,leading to pulmonary complications such as interstitial lung disease（ILD）and pulmonary hypertension（PH）.Patients who suffer from connective tissue-associated interstitial lung disease with pulmonary hypertension（CTD-ILD-PH）have a complex pathogenesis,difficult treatment,extremely poor prognosis,multidisciplinary,and no treatment guidelines for patients with CTD-ILD-PH,which has become one of the difficult clinical problems.The aim of this article is to find out the clinical characteristics and risk factors of patients with CTD-ILD-PH so that we can improve the understanding of CTD-ILD-PH.Methods:One hundred and forty-five patients with CTD who attended our hospital from October 2020 to December 2022 were retrospectively collected.145 patients were assessed for concomitant pulmonary hypertension based on systolic pulmonary artery pressure（s PAP）measured by transthoracic echocardiography（TTE）,and for concomitant interstitial lung disease based on chest HRCT,pulmonary function and respiratory symptoms,and were divided into The 145 patients were divided into the pulmonary hypertension group（CTD-PH group）,the interstitial lung disease group（CTD-ILD group）and the pulmonary hypertension with interstitial lung disease group（CTD-ILD-PH group）.The cases data were reviewed to collect basic information such as age,sex,height and weight,clinical manifestations:cough and sputum,flaky loss of teeth,chest tightness,morning stiffness,Raynaud’s phenomenon,dry eyes and mouth,lower limb oedema,hair loss,joint pain,Velcro rales,etc.Serological parameters:red blood cells（RBC）,interleukin-6（IL-6）,white blood cells（WBC）,ANA antibody titre,haemoglobin（HGB）,tumour necrosis factor alpha（TNF-α）,platelets（PLT）,triglycerides（TG）,creatinine（Cr）,D-dimer（D-Dimer）,total bilirubin（TBIL）,amino-terminal brain natriuretic peptide precursor（NT-pro BNP）,immunoglobulin G（Ig G）,CD4+lymphocytes,CD8+lymphocytes and other relevant data.ECG:presence of right deviation of the electrical axis,pulmonary P waves,cis-clock rotation,pulmonary function:total lung volume（TLC）,first second exertional expiratory volume（FEV1）,residual air volume（RV）,exertional spirometry（FVC）,carbon monoxide diffusion volume（DLCO）,etc.TTE:right ventricular internal diameter,pulmonary artery diameter,tricuspid regurgitation velocity（TRV）,systolic pulmonary artery pressure（s PAP）,HRCT of the chest:involved lung lobes,imaging manifestations.The clinical characteristics of patients with CTD-ILD-PH were analysed bycomparing data on basic information,clinical presentation,serological tests,chest HRCT,pulmonary function and cardiac ultrasound in the three groups.Results:（1）General information:mean age of the CTD-ILD-PH group was 54.23±15.68years,9 males（17.3%）,43 females（82.7%）,mean body mass index BMI 21.73±3.63kg/m2,mean age of the CTD-ILD group was 56.48±13.38 years,10 males（18.5%）,44females（81.5%）,with a mean body mass index BMI of 23.66±3.91 kg/m2,and the CTD-PH group had a mean age of 44.3±17.45 years,2 males（5.1%）and 37 females（94.9%）,with a mean body mass index BMI of 21.39±4.04 kg/m2.There was no significant difference in gender between the three groups statistically,and there were significant difference in age and BMI statistically,with the age in the CTD-ILD-PH and CTD-ILD groups being higher than the CTD-PH group;the BMI in the CTD-PH and CTD-ILD-PH groups being smaller than the CTD-ILD group.（2）Clinical manifestations:cough and sputum,Velcro rales,Raynaud’s phenome-non and lower limb oedema were statistically different among the three groups in general;cough and sputum,Velcro rales in CTD-PH group were less than CTD-ILD-PH group and CTD-ILD group;lower limb oedema in CTD-ILD group was less than CTD-ILD-PH group and CTD-PH group;Raynaud’s phenomenon in CTD-ILD-PH group was higher than CTD-ILD group;while chest tightness and shortness of breath,flaky tooth loss,dry mouth and eyes,morning stiffness,joint pain and hair loss were not statistically different overall among the three groups.（3）Primary illness:In the CTD-ILD-PH group,there were 25 cases（48.1%）of systemic lupus erythematosus（SLE）,10 cases（19.2%）of systemic sclerosis（SSc）,9cases（17.3%）of polymyositis/dermatomyositis（PM/DM）,3 cases（5.8%）of primary Sjogren’s syndrome（p SS）,2 cases（3.8%）of undifferentiated connective tissue disease（UCTD）,2 cases（3.8%）of ANCA associated vasculitis（AAV）（3.8%）,1 mixed connective tissue disease（MCTD）case（1.9%）,0 rheumatoid arthritis（RA）case（0%）;in the CTD-ILD group,there were 27 PM/DM cases（50%）,9 SLE cases（16.7%）,8AAV cases（14.8%）,4 RA cases（7.4%）,3 UCTD cases（5.6%）,2 SSc cases（3.7%）,1p SS case（1.9%）,0 MCTD case（0%）;in the CTD-PH group,there were 28 SLE cases（71.8%）,6 SSc cases（15.4%）,3 p SS cases（7.7%）,1 UCTD case（2.6%）,1 RA case（2.6%）,0 PM/DM case（0%）,0 MCTD case（0%）,0 AAV case（0%）.There were statistical differences in the composition ratios of the three groups,with the proportion of primary disease being SLE in the CTD-ILD group being much lower than that in the CTD-PH and CTD-ILD-PH groups,and the proportion of PM/DM being much higher than that in the CTD-PH and CTD-ILD-PH groups,while the remaining primary diseases were not statistically different.（4）Serological examination:NT-pro BNP,WBC,neutrophils（Neu）,RBC and HGB were statistically different overall between the three groups,with WBC,Neu,RBC and HGB levels lower in the CTD-PH group than in the CTD-ILD-PH and CTD-ILD groups;NT-pro BNP was lower in the CTD-ILD group than in the CTD-PH and CTD-ILD-PH groups.And PLT,Lym,ESR,CRP,AST,ALT,TBIL,total protein,albumin,Cr,uric acid,total cholesterol（TC）,TG,D-Dimer,fibrinogen,ANA antibody titer,lupus anticoagulant,TNF-α,IL-6,CD4+lymphocytes,CD8+lymphocytes,CD4+/CD8+,Ig G between the three groups no statistically significant difference overall.（5）Chest HRCT:Pulmonary artery widening,honeycomb and striated shadow were statistically different among the three groups overall,with the CTD-ILD group having less pulmonary artery widening than the CTD-PH and CTD-ILD-PH groups;in the CTD-ILD group less than the CTD-ILD-PH group;and striated shadow in the CTD-ILD-PH group less than the CTD-ILD group.In contrast,there was no statis-tical difference between the three groups in terms of lobe involvement,lattice-like,patchy,solid,ground glass,nodular or cystic shadows.（6）TTE:Pulmonary artery diameter,s PAP,right intraventricular diameter and TRV were statistically different overall between the three groups,pulmonary artery diameter in the CTD-ILD group less than the CTD-PH and CTD-ILD-PH groups;right intraventricular diameter in the CTD-ILD group less than the CTD-ILD-PH group;s PAP and TRV in these three groups were CTD-PH group>CTD-ILD-PH group>CTD-ILD group.（7）Pulmonary function:FVC,FEV1,FEV1%pred,RV%pred and TLC%pred were different among the three groups overall statistically,with FVC,FEV1 and FEV1%pred in the CTD-PH group being greater than those in the CTD-ILD-PH group;TLC%pred and RV%pred in the CTD-PH group being greater than those in the CTD-ILD-PH group and CTD-ILD group;while statistical same overall between the three groups of FEV1/FVC,RV,TLC,RV/TLC,DLCO,DLCO%pred.（8）ECG:statistical same between the three groups in terms of electrical axis right deviation,pulmonary P waves,and cis-clock rotation.Conclusion:1.CTD-ILD-PH patients are older,have a lower BMI,a higher proportion of SLE primary disease and a lower proportion of PM/DM primary disease,have significant cough and sputum,Velcro rales,Raynaud’s phenomenon,and higher leukocyte and neutrophil levels and NT-pro BNP levels.2.HRCT showed widened pulmonary arteries,honeycomb,high pulmonary artery diameter and right intraventricular diameter on TTE were suggestive of PH.Low pulmonary function FVC,TLC%pred,RV%pred were suggestive of ILD.3.Pulmonary artery widening on chest CT,pulmonary artery diameter on cardiac ultrasound and Raynaud’s phenomenon are risk factors for secondary PH;cough and sputum and Neu are risk factors for secondary ILD,Neu are independent risk factors. |
