Clinical Features And Follow-up Study Of Takayasu Arteritis

Part 1:Cardiac valve involvement in Takayasu arteritis:clinical features and follow-upBackground and objectiveTakayasu arteritis(TA)is a kind of rare,chronic,and nonspecific large-vessel vasculitis,which mainly involves the aorta and its main branches.Inflammation in the arterial wall causes rupture and proliferation of the elastic lamina and results in artery stenosis and dilation.TA can involve the aortic root or cardiac valves.With a prevalence of 20%-44%,aortic regurgitation is a common complication in patients with TA.Aortic regurgitation can lead to chronic heart failure and even death,and proper management may improve the prognosis of patients with aortic regurgitation due to TA.Severe aortic regurgitation requires surgical treatment.Systemic investigation with large sample size of cardiac valve dysfunction secondary to TA is rare.Only a few large clinical centers retrospectively analyzed the long-term surgical outcomes of patients with TA.We aimed to describe the prevalence,clinical characteristics,and outcomes of TA patients with cardiac valve dysfunction in a single center.MethodsWe retrospectively analyzed the clinical data of 1069 consecutive.patients with TA admitted to Fuwai Hospital from January 1992 to August 2017.We evaluated the structure and function of heart value mainly by cardiac ultrasound.Clinical characteristics were compared between patients with and without cardiac valve involvement.We collected follow-up data by regular reexamination and telephone interviews.A composite adverse event included mild or severe paravalvular leak on echocardiography,valve dysfunction,valve detachment,anastomotic aneurysm formation,new residual ascending aorta aneurysm,and death or reoperation due to postoperative complications.ResultsAmong the 1069 patients,373(34.9%)had valve involvement.Compared with patients without valve involvement,patients with valve involvement had later symptom onset(28.1±10.6 vs.25.9±9.8 years,P=0.036)and longer clinical duration(65.9 vs.50.6 months,P=0.009).Compared with patients without valve involvement,patients with valve involvement were more likely to have elevated erythrocyte sediment rate(47.4%vs.30.5%)and elevated C-reactive protein(40.1%vs.25.1%).260 patients(69.7%)had aortic regurgitation,followed by mitral regurgitation(39.1%),tricuspid regurgitation(34.6%),and pulmonary regurgitation(11.8%).Cardiac valve stenosis is rare(10 patients).Among patients with aortic regurgitation,49.6%had moderate to severe aortic regurgitation.Most other valve insufficiency was mild.Compared with patients with mild aortic regurgitation,patients with moderate to severe aortic regurgitation were more likely to have dilated ascending aorta,palpitation,dyspnea,and late symptom onset.Misdiagnosis of other cardiac diseases occurred in 11 patients,and 8 were misdiagnosed with rheumatic heart disease.41 patients(25 women and 16 men)with aortic regurgitation underwent operation in our center.23 were treated by aortic valve replacement(AVR)and 18 by composite graft replacement(CGR).The mean age at operation was 40.3 years,and median clinical duration from symptom onset to operation was 18.0 months(range,20 days to 27 years).No in-hospital death occurred.During a median follow-up of 6.6 years(interquartile range,0.6-9.4),we observed 10 composite events in the AVR group and 2 composite events in the CGR group.The unadjusted 5-year event-free rate was 55.8%and 91.7%in the AVR and CGR group,respectively(log-rank P<0.05).After adjustment for male sex,use of antiinflammatory therapy,and active disease stage at operation,CGR was associated with nominally lower events on adjusted analysis(hazard ratio,4.212;95%confidence interval[0.910,19.509];P=0.066).Conclusions(1)With a prevalence of 34.9%,cardiac valve insufficiency was common in Chinese TA patients.Aortic regurgitation was the most common involved pattern(69.7%).(2)Patients should undergo echocardiography once TA is diagnosed.In young women of reproductive age,TA should be considered when aortic regurgitation is detected.(3)About 5%of patients with TA required surgical treatment because of aortic regurgitation.CGR was a choice.Part II:Anemia in Takayasu arteritis:prevalence,clinical features,and treatmentBackground and objectiveTakayasu arteritis(TA)is a rare,chronic vasculitis,which mainly involves the aorta and its main branches.Anemia is a common comorbidity of patients with TA.Anemia is related to the prognosis and the quality of life in many diseases.Previous studies related to anemia among TA patients were of small sample size.This study aimed to describe the prevalence,clinical characteristics,and treatment in TA patients with anemia.MethodsThis retrospective study included the consecutive patients hospitalized for TA from January 2009 to April 2018.Anemia was diagnosed on the basis of hemoglobin level,according to World Health Organization criteria.Comparisons of clinical features and treatment were made according to the presence of anemia.Follow-up data were collected from the electronic medical records.ResultsAmong the 533 patients with TA,194 patients(36.4%)were anemic.Female patients were predominant(92.8%).Age at symptom onset and age at evaluation were similar with respect to anemia,but anemic patients had a shorter clinical duration(54.8 vs.79.4 months,P=0.039).Compared with non-anemic patients,anemic patients were more likely to have active disease stage(64.9%vs.50.1%)and dizziness(29.4%vs.21.2%).Pulmonary artery involvement(12.4%vs.26.8%),pulmonary hypertension(12.9%vs.20.1%)and use of pulmonary hypertensive-target drugs(2.8%vs.11.6%)were less common among anemic patients than among non-anemic patients(all P<0.05).A total of 177 patients(91.2%)had mild anemia.Normocytic anemia(62.9%)was the most common pattern.Lower body mass index and lower left ventricular ejection fraction were observed in anemic patients.The proportion of patients with prednisone was similar with respect to anemia,but anemic patients received a higher dose of prednisone.35 patients received oral iron supplementation.Over a median follow-up of 4.0 months(interquartile range,2.1-11.8),43.7%of anemic patients remained anemic.The difference was not significant between patients with(44.8%)and without(42.0%)iron supplementation.Compared with patients without iron supplementation,hemoglobin increased more among patients with iron supplementation.Conclusions(1)With a prevalence of 36.4%,anemia was a common concurrent condition in TA,especially in young,female patients.(2)Active disease stage was more common in anemic patients.(3)Iron supplementation may help increase hemoglobin.Part ?:Tuberculosis in Takayasu arteritis:prevalence,clinical features,and treatmentBackground and objectiveTakayasu arteritis(TA)is a rare,chronic,granulomatous vasculitis,which mainly involves the aorta and its main branches.Coronary arteries and pulmonary arteries can also be involved.Tuberculosis(TB)has been reported to be a trigger of TA.Pathological characteristics of both TA and TB are granulomas and caseous necrosis.TB and infection of Mycobacterium tuberculosis are more prevalent in patients with TA than in the general population.We aimed to describe the proportion,clinical characteristics and prognosis of TA patients with TB.MethodsWe included 1105 patients with TA,who were hospitalized between January 1992 and December 2017.Comparisons of clinical features and treatment were made according to the presence of TB.We also analyzed the outcomes of TA patient with active TB.ResultsAmong the 1105 patients,109(9.9%)had TB.Among patients with TB,the female to male ratio was 3.7:1.The mean age at symptom onset was 26.7±10.0 years.The median duration from symptom onset to admission was 56.3 months.The demographic features were similar with respect to TB.Compared with patients without TB,active disease stage(61.5%vs.48.4%),constitutional symptoms(15.6%vs.8.7%),hemoptysis(7.3%vs.1.4%),pulmonary hypertension(19.3%vs.10.7%),and pulmonary artery involvement(31.2%vs.17.3%)were more common among patients with TB.Pulmonary TB was most frequently identified(97 patients,89.0%),followed by TB lymphadenitis(8 patients,7.3%).53 patients(48.6%)were diagnosed with TB before the onset of TA,23(21.1%)with a concurrent diagnosis of TB and TA,and 24 patients(22.0%)developed TB after TA.The use of steroids was similar between patients with and without TB,but interventional treatment was less common in patients with TB.Ten patients with active TB received triple anti-tuberculosis medication,and 3 patients initiated prednisone.We did not observe TB diffusion during follow-up.Conclusions(1)The proportion of Chinese TA patients with TB was not low,and about half of them developed TB before TA.Pulmonary TB was the most common pattern.(2)Pulmonary artery involvement,pulmonary hypertension,and active disease stage were more frequent in TA patients with TB.(3)TB should be screened before initiating anti-inflammatory therapy.Close monitor of inflammation and TB was necessary.