Clinical Analysis And Risk Factors Of Mixed Connective Tissue Disease Associated Pulmonary Artery Hypertension Involvement

Objective:The clinical data of 105 patients with mixed connective tissue disease were retrospectively analyzed,aimed to understand the clinical characteristics of MCTD patients,and study the risk factor of mixed connective tissue disease(MCTD)associated intesitial lung disease(ILD)/pulmonary artery hypertension(PAH).Methods: A total of 105 patients diagnosed as mixed connective tissue disease and regularly followed up in the department of rheumatology and immunology,Peking University shenzhen hospital from January 2008 to August 2019 were selected.A total of 94 patients were followed up regularly for more than 1 year.Clinical data werecollected,including general situation,clinical manifestations,laboratory examination and imaging examination results.And the data were retrospectively analyzed.Results:(1)Among the 105 patients included,male: female =1:9.5,with more females.The mean age of onset was 36.88±12.88 years,and the peak age was 30-40 years.The disease course was 5.0(2.3,8.5)years.Raynaud's phenomenon(37.14%)was the most common original symptom,followed by synovitis(36.19%).92.38% of the patients developed Raynaud's phenomenon,87.62% developed synovitis,76.19% developed interstitial lung disease,and 7.62% developed pulmonary hypertension.The ANA karyotype was mainly granular in 105 patients(99.05%).(2)All 105 patients were highly titer positive against U1 RNP.The incidence of interstitial lung disease,pericardial effusion and dry eyes in the anti-SSA antibody positive group was higher than that in the anti-SSA antibody negative group(P < 0.05),and the incidence of myasthenia was lower than that in the anti-SSA antibody negative group(P < 0.05).The incidence of PAH in the anti-RO-52 positive group was higher than that in the antiRO-52 negative group(P < 0.05).(3)The onset age of the ILD group was higher than that of the non-ILD group(P < 0.05).The incidence of myasthenia in the ILD group was lower than that in the non-ILD group(P < 0.05).The positive rate of anti-SSA antibody in the ILD group was higher than that in the non-ILD group(P < 0.05).Age of onset(RR=1.026;95%CI=1.008?1.044;P=0.005)and anti-SSA antibodies(RR=2.156;95%CI=1.283 ? 3.622;P=0.004)were independent risk factors for MCTD-ILD.The ILD appeared later in patients with myasthenia than in patients without myasthenia.(4)There were no significant differences in gender,age of onset and course between PAH group and non-PAH group(P >0.05).The incidence of pericardial effusion and heart failure in PAH group was higher than that in non-PAH group(P < 0.05).The positive rate of anti-RO-52 antibody in PAH group was higher than that in non-PAH group(P <0.05).Hs-CRP level in PAH group was higher than that in non-PAH group(P < 0.05).The serum uric acid level of PAH group was higher than that of non-PAH group(P <0.05).The HDL-C level of PAH group was lower than that of non-PAH group(P <0.05).Serum uric acid(RR=1.029;95%CI=1.005?1.053;P=0.018)is an independent risk factor for MCTD-PAH,while HDL-C(RR=0.0001;95%CI=0.000?0.268;P=0.022)is MCTD-PAH Protective Factor.Conclusion: 1.MCTD tends to occur in young women,with the primary original symptoms being Raynaud's phenomenon and arthritis,and interstitial lung disease being the more common clinical manifestations.ANA karyotype in MCTD patients was mainly granular.2.Anti SSA antibody is associated with ILD,pericardial effusion,dry eyes,muscle weakness in MCTD patients.Anti-RO-52 antibody is associated with PAH in MCTD patients.3.Older with positive anti-SSA antibodies MCTD patients have an increased risk of developing ILD.4.MCTD patients with elevated uric acid levels have an increased risk of PAH,and serum HDL-C may be a protective factor for MCTD-PAH.