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Objective To determine the clinical significance of pulmonary function test(PFT)in evaluating the features and severity of lung impairments associated with connective tissue diseases(CTD)by comparing the differences of pulmonary function test parameters among groups of CTD associated pulmonary disorders.Methods Cases of CTD associated pulmonary disorders were prospectively enrolled and assigned into 3 groups according to their lung impairments:CTD associated pulmonary arterial hypertension group (CTD-PAH,n=29),CTD associated interstitial lung disease group(CTD-ILD,n=35),CTD associated PAH plus ILD group(CTD-PAH+ILD,n=16)and CTD control group(n=34).Pulmonary function test parameters,including total lung capacity(TLC % predicted),forced vital capacity(FVC % predicted),forced expiratory volume in the first second(FEV_(1.0)% predicted),FE_(1.0)%/FVC and diffusing capacity of the lung for carbon monoxide(DLco,% predicted)were measured and compared among the four groups.Results One hundred and forteen eases were included and predominantly female with average onset age of 35～39 years old.CTDs that were predisposed to lung diseases were mixed connective disease(MCTD),systemic sclerosis(SSc),systemic lupus erythematosus(SLE)and primary Sj(o)ren syndrome(pSS),in order.There were 10,29 and 46 percent of patients presented with decreased TLC% in CTD-PAH,CTD-ILD and CTD-PAH+ILD group respectively,50,36 and 71 percent of patients with decreased FVC% respectively,54,47 and 71 percent of patients with decreased FEV_(1.0)% respectively,and 100,82 and 100 percent with decreased DLco% respectively.ANOVA analysis demonstrated that TLC%,FVC%,FEV_(1.0)%,DLco% had significant differences between CTD control group and each of the CTD associated lung disease group(P＜0.05),although none of them was lack of difference between the PAH and ILD groups.TLC% was significantly higher in CTD-PAH group than CTD-PAH+ILD group[(89±15)% vs(79±12)%,P＜0.05],while FVC% was significantly lower in CTDPAH+ILD group either than CTD-PAH group or than CTD-ILD group[(81±13)%,(80±16)% vs(65±22)%,P＜0.05].ConclusionPulmonary function test may be valuable in early screening for CTD associated lung disorders than distinguishing CTD-PAH from ILD,which usually reveal restrictive ventilation dysfunction and/or diffusing capacity dysfunction.
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Objective To analyze the clinical characteristics of large pulmonary vessel involvement in Behcet's diseaseMethods The clinical data of Behcet's disease patients with large pulmonary vessel involvement,who were treated in Peking Union Medical College Hospital from 1983 to 2008,were retrospectively analyzed.Results Thirteen patients had large pulmonary vessel involvement among a total of 418 patients with Behcet's disease.The 13 patients had a mean age of (37.5?12)years old,with 10(69.2%)having hemoptysis,8(61.5%)having dyspnea.Helical CT/CTPA showed that pulmonary vascular involvement included pulmonary arterial aneurysms(53.8%),pulmonary artery occlusion(46.2%),and pulmonary thrombi(46.2%).All the 13 patients were treated with steroid and immunosuppressant;five received steroid pulse therapy;and four received vascular intervention therapy.The mean follow-up was (3?1.6)years,with a follow-up rate of 92.5%.The 3-year survival rate was 83.9% for the 13 patients.Conclusion The manifestation of pulmonary vascular involvement in Behcet's disease is various,and the diagnosis mainly depends on the manifestations of CT pulmonary angiography.
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Objective To investigate the clinical features with to highlight the main criteria for diagnosis of portopulmonary hypertension(POPH).Methods The clinical and laboratory data of 7 patients with POPH were retrospectively analysed.Results The patients included 2 males and 5 females with median age at diagnosis of 51 years(ranging from 26 to 73 years).The underlying diseases of portal hypertension were post-type B hepatitis cirrhosis(3 cases),autoimmune hepatitis(2 cases),immunologic deficiency disease(1 case)and portal vein thrombosis(1 case).The initial symptom of pulmonary hypertension(PH)in 5 patients was dyspnea on exertion,but 2 patients remain asymptomatic.All patients was assessed by transthoracic echocardiogram(TTE)with the pulmonary arterial systolic pressure(PASP)above 55 mmHg,1 patient accepted right heart catheterization(RHC)with mean pulmonary arterial pressure of 62 mmHg and pulmonary vascular resistance of 8.64 mN/(s?cm5).Immunologic abnormality was found in 6 patients,and the severity of liver function does not appear to correlate with the severity of the PH.Follow-up study demonstrated 3 patients died,2 of whom were due to the cardiac involvement(right heart failure and sudden death).Conclusion PH is a rare but severe complication of portal hypertension.It is the portal hypertension,not the underlying liver disease causing POPH.TTE is an important screening procedure,but the gold standard for diagnosis of POPH is hemodynamic data from RHC.
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Objective To investigate the diagnostic status of pulmonary thromboembolism (PTE) for inpatients in Peking Union hospital Methods Inpatients diagnosed acute PTE from January 1999 to December 2002 were reviewed and divided by sex, age and risk factors Results Among 78 844 inpatients, 155 were diagnosed PTE during 4 year period, 73 of 27 100 in men (0 27%) vs 82 of 51 744(0 16%)in women, and 52 of 17 459 (0 30% ) in elderly patients(≥60 years) vs 103 of 61 385 (0 17% ) in non elderly patients(