Clinical Analysis Of 80 Cases Of Primary Sj(?)gren's Syndrome With Pulmonary Arterial Hypertension

Objective To analyze the prevalence and risk factors of primary Sj(?)gren's syndrome(pSS)with pulmonary artery hypertension(PAH)for early diagnosis and treatment.Methods 438 patients with pSS from August 2009 to March 2019 in Anhui Provincial Hospital were selected and analyzed retrospectively according to the diagnostic criteria of pulmonary hypertension(pulmonary artery pressure was estimated by transthoracic Doppler echocardiography and positive results were? 40 mm Hg).Results Of the 438 patients with pSS,80 had PAH,the pulmonary artery pressure was between 40 and 114 mm Hg.The incidence of pSS-PAH was 18.3%,of which 75(93.8%)were females,aged 22 to 77(59.9 ± 13.8)years old.The most common initial symptom were dryness(dry mouth and / or dry eyes)in 37 cases(46.3%),followed by pulmonary symptoms in 24 cases(30.0%),joint pain in 15 cases(18.8%),and tooth loss in 5 cases(6.3%),4 cases of rash(5.0%),4 cases of Raynaud's phenomenon(5.0%).All nine factors that may cause pSS with PAH(P<0.05),including the initial symptom of pulmonary manifestations,interstitial pneumonia,pericardial effusion,age,serum albumin,complement C3,left atrial diameter,Left Ventricular End-Systolic Dimension,Schirmer test were included in the multivariate Logistic regression analysis.The results showed that the three variables were statistically significant,interstitial pneumonia(OR 3.387,P = 0.002),age(OR 1.040,P=0.005),and initial lung symptoms(OR 3.104,P=0.010)were independent risk factors for pSS-PAH.Conclusion Pulmonary hypertension is not uncommon in primary Sj(?)gren's syndrome.interstitial pneumonia,age,and initial pulmonary symptoms are related to their occurrence.Echocardiography and further examination should be performed for early diagnosis and treatment,which can improve the prognosis of patients.