Clinical Feature And Pathogenesis Study Of Takayasu's Arteritis

Objective:The aim of this study was to evaluate the efficacy and safety of tocilizumab(TCZ)in Chinese Takayasu' s arteritis(TAK)patients.Methods:This was a single center,prospective study.White blood cell(WBC),hemoglobulin(Hb),erythrocyte sedimentation rate(ESR)and hyper-sensitive C reactive protein(hsCRP)were recorded at baseline and before each TCZ infusion.Doppler Ultrasonography was used to track vascular changes every 6 months during study.This study was approved by the Institution of Review Board(IRB)of Peking Union Medical College Hospital(No.S-478)and registered in Chinese Clinical Trial Registry system(ChiCTR-OPC-16009231).Results:Sixteen patients with a median age of 26.5(18-47)years were recruited and analyzed.One patient was treatment naive;the others had taken a median of 3(1-5)conventional immune suppressants before TCZ therapy.Three patients withdrew TCZ after 1 infusion each due to unbearable neck pain.The other 13 patients were treated with TCZ for a median of 13(7-20)months.After TCZ treatment,the median ESR level,hsCRP level,mural thickness of common carotid artery and subclavical artery decreased from 39(7-92)mm/h,28.88(7.6-155.93)mg/L,0.24(0.06-0.59)cm,0.18(0.07-0.47)cm to 6(1-30)mm/h(P=0.000),0.59(0.08-19.12)mg/L(P=0.006),0.17(0.04-0.53)cm(P =0.000),and 0.12(0.07-0.18)cm(P=0.035),respectively.The glucocorticoid dosages were tapered or maintained in all patients.One episode of urinary infection was recorded and relieved after antibiotic therapy.Neither neutropenia nor abnormal liver enzyme was observed.Conclusion:Our study suggests that TCZ is a safe and effective agent for long term use among TAK patients.Objective:The aim of this study was to analyze the relationship between serum pentraxin-3(PTX-3)/lysosomal associated membrane protein-2(LAMP-2)levels and disease activity in Takayasu's arteritis(TAK).Methods:All patients' clinical symptoms,physical findings,auxiliary examinations and image results were detailedly recorded in the Chinese Rheumatism Data Center(CRDC)database.The disease activity was defined according to the National Institute of Health(NIH)criteria.Serum PTX-3/LAMP-2 levels were measured by commercial enzyme-linked immuno sorbent assay(ELISA)kits under the manufacturers' guidance.Thereafter,the relationship between serum pentrixin-3(PTX-3)/lysosomal associated membrane protein-2(LAMP-2)level and disease activity in TAK was analyzed.This study was approved by the Institution Review Board(IRB)of Peking Union Medical College Hospital(PUMCH)and written informed consent was obtained from each patient.Results:A total of 98 TAK patients and age/gender matched healthy controls(HCs)were enrolled and analyzed in the present study.Eighty seven of the 98 TAK patients were women and forty five patients had active disease.The average age at disease onset and disease duration were 27.12±9.23 years and 46.53 ± 59.28 months,respectively.Compared with HCs,patients with TAK had higher serum PTX-3 and LAMP-2 levels(PTX-3:0.32±0.23 ng/ml VS 0.18±0.09 ng/ml,P=0.001;LAMP-2:4.80±2.10ng/ml VS 3.30±1.25ng/ml,P<0.001).Patients with active disease had higher serum PTX-3 levels compared with those who had inactive disease(0.39±0.28 ng/ml VS 0.26±0.16 ng/ml,P=0.004).However,no statistically significant difference was observed between patients with and without active disease in serum LAMP-2 levels.With established threshold at 0.3071 ng/ml,PTX-3 had a sensitivity of 60.00%and a specificity of 79.25%for diagnosing active TAK.When combined with erythrocyte sedimentation rate(ESR)/hyper-sensitive C reactive protein(hsCRP),the specificity of PTX-3 for diagnosing active TAK raised to 90.30%.Conclusion:Compared with HCs,TAK patients had higher serum PTX-3 and LAMP-2 levels.TAK patients with acitve disease had higher serum PTX-3 but not LAMP-2 levels compared with pattients who had inactive disease.Objective:To investigate the T helper cell(Th)subgroup proportions in peripheral blood mononuclear cells(PBMCs)of Takayasu' s arteritis(TAK).And to analyze the relationship between Th proportions and TAK disease activity.Methods:Samples were collected from TAK patients and age/gender matched healthy controls(HCs)in Peking Union Medical College Hospital,a Chinese nationwide referral center,from January 2017 to April 2017.TAK disease activity was assessed according to the National Institutes of Health(NIH)criteria.The proportions of Ths in PBMCs were measured by the flow cytometry.Results:A total of 10 TAK patients and 5 gender/age matched HCs were recruited and analyzed in the current study.Among the 10 TAK patients,8 of them were women,3 were with active disease and 4 were treatment naive.Compared with HCs,Th 17 and Th22 proportions were significantly increased(1.29±0.52%VS 0.72±0.21%,P=0.035;and 0.79±0.38%VS 0.36±0.12%,P=0.031;respectively).Other Th subgroups were not statistically different between TAK patients and HCs.Compared with TAK patients who had inactive disease,those with active disease had higher Thl and Th9 proportions.However,the differences didn' t reach statistical level(18.53 ± 8.10%VS 11.02±3.24%,P=0.059;0.38±0.20%VS 0.17±0.15%,P=0.098;respectively).Th17 proportions in PBMCs were positively correlated with erythrocyte sedimentation rate(ESR)and serum hyper-sensitive C reactive protein(hsCRP)levels(r =0.624,P=0.045 and r=0.642,P=0.045).While no statistical correlation was observed between Th22 proportions and ESR/hsCRP.Conclusions:Th17 and Th22 proportions in PBMCs were elevated in TAK patients.However,no statisitcally significant difference was observed between TAK patients with active and inactive disease in Th proportions.Th17 proportions in PBMCs were positively correlated with ESR and serum hsCRP levels.Objective:Takayasu' s arteritis(TAK)is a rare systemic vasculitis which mainly affects the aorta and its major branches.Previous studies have suggested that almost half of the Asian TAK patients have renal artery involvement(RAI).We carried out this study to clarify the characteristic of RAI in Chinese TAK patients.Methods:We retrospectively reviewed and analyzed 411 patients diagnosed with TAK from 1990 to 2014 in Peking Union Medical College Hospital to explore the clinical features of RAI in this group of TAK patients.Results:A total of 201 patients were detected to have RAI.Stenosis(78.11%)was the most common RAI pattern.Compared to those without RAI,patients with RAI were younger at disease onset(23.50±9.60 VS 25.56±9.50 years,P=0.029)and more likely to have hypertension(74.63%VS 28.10%,P<0.001).Congestive heart failure(22.38%VS 7.62%,P<0.001)and pulmonary hypertension(19.90%VS 9.52%,P=0.003)were more prevalent among patients with RAI than those without.Conclusion:Stenosis is the most common RAI pattern.Patients with RAI have more severe cardiac dysfunction compared to those without.