Prevalence of Anti-endothelial Cell Antibodies in Patients with Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Objective To investigate the prevalence of anti-endothelial cell antibodies (AECAs) in the sera of connective tissue diseases (CTD) patients with pulmonary arterial hypertension (PAH) and its correlation with clinical manifestations. Methods AECAs in sera of 39 CTD patients with PAH,22 CTD patients without PAH,and 10 healthy donors as controls were detected with Western blotting. The prevalence of different AECAs in different groups was compared and its correlation with clinical manifestations was also investigated. Results The prevalence of AECAs was 82.1% in CTD patients with PAH,72.7% in CTD patients without PAH,and 20.0% in healthy donors. Anti-22 kD AECA was only detected in CTD patients with PAH (15.4%). Anti-75 kD AECA was more frequently detected in CTD patients with PAH than in those without PAH (51.3% vs. 22.7%,P<0.05). In CTD patients with PAH,anti-75 kD AECA was more frequently detected in those with Raynaud’s phenomenon or with positive anti-RNP antibody. Conclusion AECAs could be frequently detected in CTD patients with or without PAH,while anti-22 kD and anti-75 kD AECA might be specific in CTD patients with PAH.

Number and Distribution of Fiducial Fibers in a Spectroscopic Survey Telescope

To date,the Large Sky Area Multi-Object Fiber Spectroscopic Telescope(LAMOST)has been in operation for 12 yr.To improve the telescope's astronomical observation accuracy,the original open-loop fiber positioning system of LAMOST is in urgent need of upgrading.The upgrade plan is to install several fiber view cameras(FVCs)around primary mirror B to build a closed-loop feedback control system.The FVCs are 20 m from the focal surface.To reduce a series of errors when the cameras detect the positions of the optical fibers,we designed fiducial fibers on the focal surface to be fiducial points for the cameras.Increasing the number of fiducial fibers can improve the detection accuracy of the FVC system,but it will also certainly reduce the number of fiber positioners that can be used for observation.Therefore,the focus of this paper is how to achieve the quantity and distribution that meet the requirements of system detection.In this paper,we introduce the necessity of using fiducial fibers,propose a method for selecting their number and present several methods for assessing the uniformity of their distribution.Finally,we implement particle swarm optimization to find the best distribution of fiducial fibers.

Chinese Systemic Lupus Erythematosus Treatment and Research Group Registry IX： Clinical Features and Survival of Childhood-Onset Systemic Lupus Erythematosus in China

Background： Approximately 15-20% cases of systemic lupus erythematosus （SLE） are diagnosed in children. There have been a Few studies reporting the epidemiological data of pediatric-onset SLE （cSLE） in China, neither comparing the differences between cSLE and adult-onset SLE （aSLE）. The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group （CSTAR） database. Methods： We made a prospective study of 225 cSLE patients （aged 〈16 years） and 1759 patients aged 16-50 years based on CSTAR registry. We analyzed initial symptoms, clinical presentations, SLE disease activity, damages, and outcomes ofcSLE, as well as compared with aSLE patients. Results： The mean age ofcSLE patients was 12.16 ± 2.92 years, with 187 （83.1%） females. Fever （P 〈 0.001） as well as mucocutaneous （P 〈 0.001 ） and renal （P = 0.006） disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects （P 〈 0.001 ）. The eSLE patients were found to present more fi＇equently with malar rash （P = 0.001; odds ratio {OR], 0.624; 95% confidence interval [CI ], 0.470 0.829） but less tYequently with arthritis （P 〈 0.001 ; OR, 2.013; 95% CI, 1.512-2.679） and serositis （P = 0.030; OR, 1.629; 95% CI, 1.053 2.520）. There was no significant difl＇erence in SLE disease activity index scores between cSLE and aSLE groups （P = 0.478）. Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients （hazard ratio 0.335 [0.170 0.658], P = 0.001）. The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test （0.557, P = 0.455）. Conclusions： cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difl＇erence in 5- year survival between cSLE and aSLE groups.

CBLN2 rs2217560 was Associated with Pulmonary Arteria Hypertension in Systemic Lupus Erythematosus

To the Editor:Pulmonary arterial hypertension (PAH)is a hemodynamic disorder with elevated pressure of pulmonary circulation.Genetic studies in familial PAH (fPAH)and idiopathic PAH (iPAH)have discovered that transforming growth factor-β (TGF-β) superfamily plays an important role,and the identified mutations occur in bone morphogenetic protein type 2 receptor (BMPR2), activin receptor-like kinase type 1 (ALK1),Endoglin,and SMAD9. A genome-wide association study (GWAS)in patients without BMPR2 mutations discovered that one single-nucleotide polymorphism (SNP)rs2217560 had a significant association with i/fPAH,which located 52-kb downstream of the CBLN2 gene.

Chinese registry of rheumatoid arthritis: IV. Correlation and consistency of rheumatoid arthritis disease activity indices in China

Background:Disease activity indices(DAIs)including disease activity score 28(DAS28),simplified disease activity index(SDAI),and clinical disease activity index(CDAI)have been widely used in clinical practice and research studies of rheumatoid arthritis(RA).The objective of our study was to evaluate the correlation and concordance among different DAIs in Chinese patients with RA.Methods:A cross-sectional study,including patients enrolled in the Chinese registry of rheumatoid arthritis from November 2016 to August 2018,was conducted.The correlations were evaluated using Spearman correlation coefficient and concordance with Bland-Altman plots,quadratic weighted kappa,and discordance rates in the crosstab.For other indices,the optimal cutoff points corresponding to SDAI remission were explored through receiver operating characteristic curve analysis.Results:A total of 30,501 patients were included,of whom 80.46%were women.Most individuals were with moderate disease activity or high disease activity.High correlations among DAS28-erythrocyte sedimentation rate(ESR)and DAS28-C-reactive protein(CRP),SDAI and CDAI were observed.Similarly,the weighted kappa value among the indices was high.In Bland-Altman plots,a positive difference between DAS28-ESR and DAS28-CRP was observed,with an absolute difference of>1.2 in 3079(10.09%)patients.In crosstab,approximately 30%of the patients were classified into different groups.Concordance values between SDAI remission and the optimal cutoff points of DAS28-ESR,DAS28-CRP,and CDAI were 3.06,2.37,and 3.20,respectively.Conclusions:Although DAIs had high correlations and weighted kappa values,the discordance between DAIs was significant in Chinese patients with RA.The four DAIs are not interchangeable.

Treatment satisfaction with rheumatoid arthritis in patients with different disease severity and financial burden:A subgroup analysis of a nationwide survey in China

Background:Despite the recent advances in treatments for rheumatoid arthritis (RA), there are still unmet needs in disease outcomes. This study aimed to analyze the satisfaction with drug therapies for RA according to the levels of disease severity (patientassessed) and proportions of treatment cost to household income.Methods:This was a subgroup study of a cross-sectional study in patients with RA and their physicians. The patients were subdivided into different subgroups based on their self-assessed severity of RA and on the proportions of treatment cost to household income (<10%, 10-30%, 31-50%, and >50%). The Treatment Satisfaction Questionnaire for Medication version II was used to assess patients’ treatment satisfaction.Results:When considering all medications, effectiveness, convenience, and global satisfaction scores were lower in the severe and moderate RA subgroups than those in the mild and extremely mild RA subgroups (all P < 0.001). Effectiveness, side effects, and convenience scores were higher in the <10% subgroup compared to those in the >50% subgroup (all P < 0.05). Global satisfaction score was higher in the <10% subgroup than that in the 31% to 50% subgroup ( F = 13.183, P = 0.004). For biological diseasemodifying anti-rheumatic drugs, effectiveness and convenience scores were lower in the severe RA subgroup than those in the extremely mild RA subgroup (both P < 0.05). Convenience score was higher in the <10% subgroup compared to that in the 31% to 50% and >50% subgroups ( F = 12.646, P = 0.005). Global satisfaction score was higher in the <10% subgroup than that in the 31% to 50% subgroup ( F = 8.794, P = 0.032). Conclusion:Higher disease severity and higher financial burden were associated with lower patient satisfaction.

Lupus Myocarditis： A Case-Control Study from China

Background：Myocarditis is an uncommon but serious manifestation of systemic lupus erythematosus （SLE）.This study aimed to investigate clinical characteristics and outcomes of lupus myocarditis （LM） and to determine risk factors of LM in hospitalized Chinese patients with SLE.Methods：We conducted a retrospective case-control study.A total of 25 patients with LM from 2001 to 2012 were enrolled as the study group,and 1 O0 patients with SLE but without LM were randomly pooled as the control group.Univariable analysis was performed using Chi-square tests for categorical variables,and the Student＇s t-test or Mann-Whitney U-test was performed for continuous variables according to the normality.Results：LM presented as the initial manifestation of SLE in 7 patients （28％） and occurred mostly at earlier stages compared to the controls （20.88 ± 35.73 vs.44.08 ± 61.56 months,P =0.008）.Twenty-one patients （84％） experienced episodes of symptomatic heart failure.Echocardiography showed that 23 patients （92％） had decreased left ventricular ejection fraction （＜50％） and all patients had wall motion abnormalities.A high SLE Disease Activity Index was the independent risk factor in the development of LM （odds ratio =1.322,P ＜ 0.001）.With aggressive immunosuppressive therapies,most patients achieved satisfactory outcome.The in-hospital mortality was not significantly higher in the LM group than in the controls （4％ vs.2％,P =0.491）.Conclusions：LM could result in cardiac dysfunction and even sudden death.High SLE disease activity might potentially predict the occurrence of LM at the early stage of SLE.Characteristic echocardiographic findings could confirm the diagnosis of LM.Early aggressive immunosuppressive therapy could improve the cardiac outcome of LM.