[Show abstract][Hide abstract] ABSTRACT: Our study aimed to investigate the effect of cigarette smoking on the clinical phenotype of patients registered in the Chinese Systemic Lupus Erythematosus (SLE) Treatment and Research (CSTAR) group registry database, the first online registry of Chinese patients with SLE.
A prospective cross-sectional study of Chinese SLE patients was conducted using the CSTAR. Our case-control analysis was performed on age- and gender-matched subjects to explore the potential effect of cigarette smoking on the clinical manifestation of SLE.
Smokers comprised 8.9% (65/730) of patients, and the ratio of females/males was 19/46. Thirty-nine patients were current smokers, and 26 were ex-smokers. Data showed significant differences between smokers and nonsmokers in the following areas: nephropathy (58.5% vs. 39.2%; p = 0.003), microscopic hematuria (30.8% vs. 19.1%; p = 0.025), proteinuria (53.8% vs. 34.4%; p = 0.002), and SLE Disease Activity Index(DAI) scores (12.38±8.95 vs. 9.83±6.81; p = 0.028). After adjusting for age and gender, significant differences between smokers and nonsmokers were found with photosensitivity (35.9% vs. 18%; p = 0.006), nephropathy (59.4% vs. 39.8%; p = 0.011), and proteinuria (54.7% vs. 35.2%). Although smokers tended to have greater disease severity compared with nonsmokers (SLEDAI scores: 12.58±8.89 vs.10.5±7.09), the difference was not significant (p = 0.081).
Cigarette smoking triggers the development and exacerbation of SLE, especially with respect to renal involvement. Chinese smokers with SLE should be advised to discontinue cigarette use.
PLoS ONE 08/2015; 10(8):e0134451. DOI:10.1371/journal.pone.0134451 · 3.23 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: This review introduces the history of development, organizational structure, funding resources, data collection, and quality control of the Chinese Rheumatism Data Center (CRDC) and summarizes the collection of data. In 2009, Peking Union Medical College Hospital (PUMCH), together with several rheumatism centers, established the Chinese Systemic Lupus Erythematosus (SLE) Treatment and Research Group (CSTAR) to collect data on Chinese patients for the study of SLE disease characteristics. In 2011, CSTAR was extended with the formation of the CRDC at PUMCH with direction from the National Health and Family Planning Commission of the PRC. The CRDC currently includes 300 registration sites and 50 regional sites that have successively begun to collect data on 12 rheumatic diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), ankylosing spondylitis (AS), systemic sclerosis, dermatomyositis, Takayasu arteritis, IgG4-related diseases, ANCA-associated vasculitis, gout, polyarteritis nodosa, unclassified systemic vasculitis, and Behcet disease. To date, 17,224 patients have been enrolled in the CRDC. Based on the SLE patients registered in the CRDC, papers investigating basic demographic characteristics and first symptom in Chinese SLE patients, risk factors of pulmonary hypertension, correlations between autoantibodies and clinical manifestations, and factors related to fetal loss have been published. The CRDC is a national registry that provides real-life data to improve clinical decision-making. At the same time, without additional work for the clinician, the CRDC is a powerful research database. The CRDC database provides sufficient information for Chinese clinical studies on rheumatology. Moreover, a mobile device application ensures convenient and efficient data collection without compromising data quality, thereby providing strong evidence-based data for the diagnosis and treatment of Chinese rheumatic patients.
[Show abstract][Hide abstract] ABSTRACT: Severe aortic regurgitation (AR) attributable to Behçet's disease (BD) is a rare but challenging problem in light of the surgical treatment notoriously complicated with paravalvular leakage (PVL) and high mortality. This study aims to test whether immunosuppressive therapy is effective in reducing the complication of the operation and improve the prognosis.
In a retrospective cohort of 644 hospitalised BD patients who were diagnosed and followed up between January 1998 and September 2014, 18 patients (2.8%) with severe AR due to BD were identified and their medical records were analysed.
Among the 18 patients, 15 patients underwent the valve surgery and PVL developed in 7 (47%) at a median interval of 3.5 months. In the median follow-up of 32.5 months from first operations, patients with PVL had a significantly higher incidence of repeat surgery and death (71% vs. 0%, p=0.007). They less likely received preoperative immunotherapy (14% vs. 100%, p=0.001), had a lower cumulative dosage of cyclophosphamide before first operations (2.1±5.7g vs. 13.0±6.4g, p=0.004) and a higher preoperative erythrocyte sedimentation rate (44.4±20.7mm/first hour vs. 25.0±12.1mm/first hour, p=0.04) compared with those without developing PVL. Multivariate analysis showed preoperative immuno-suppressive therapy (hazard ratio 18.58; 95% confidence interval, 2.134-161.81; p=0.008) was an independent factor associated with the absence of PVL. The 5-year PVL-free survival rates were significantly higher in patients receiving preoperative immunotherapy (p=0.0004).
In patients with severe AR due to BD, preoperative immunosuppressive therapy, especially cyclophosphamide in conjunction with glucocorticoid, could reduce PVL after the corrective surgery and improve the outcomes.
Clinical and experimental rheumatology 05/2015; · 2.72 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To investigate the prevalence and clinical relevance of telangiectasia in Chinese patients with systemic sclerosis (SSc).
Data from 230 SSc EUSTAR patients from Peking Union Medical College Hospital (2009-2011) that fulfilled the 1980 American College of Rheumatology SSc classification criteria were prospectively collected. Demographic, clinical, and laboratory data were calculated between groups with and without telangiectasia, and a six-minute walk test, pulmonary function test (PFT), transthoracic echocardiography (TTE), right heart catheterisation (RHC) and modified Rodnan skin score (mRSS) were performed.
96 patients (41.7%) were diagnosed with telangiectasia. There were no significant differences between patients with and without telangiectasia based on gender, age at onset, Raynaud's phenomenon (RP) duration, or SSc classification. Disease duration both from RP onset of patients and from first non-RP manifestation of patients with telangiectasia was significantly longer than patients without (p<0.05). RP (97.9% vs. 90.3%), finger/toe sclerosis (96.9% vs. 88.1%), facial sclerosis (68.8% vs. 53.7%), digital ulcers (DUs; 40.6% vs. 23.1%), digital pitting (49.0% vs. 33.8%), joint contracture (20.8% vs. 10.4%) and erythrocyte sedimentation rate elevation (26.7% vs. 14.8%) were significantly greater in telangiectasia patients (p<0.05). There were no differences in autoantibody development between patients with and without telangiectasia (p>0.05). PFT showed that forced vital capacity (77.0±17.26 vs. 83.05±16.53, p=0.005) and diffusion capacity for CO of the lung (58.9±19.4 vs. 65.7±19.7, p=0.030) were lower, while forced expiratory volume ratio (87.02±7.8 vs. 84.33±7.1, p=0.029) was higher in SSc with telangiectasia. Pulmonary artery hypertension (PAH) prevalence (25.0% vs. 14.2%) was significantly greater in patients with telangiectasia.
Telangiectasia are common in Chinese SSc patients and usually associated with DUs, RP, and PAH. Telangiectasia could be a clinical marker of microvascular disease in SSc.
[Show abstract][Hide abstract] ABSTRACT: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with an increased risk of death compared to general population. Although previous studies showed improvement in survival of SLE, the long-term prognosis has not been elaborated in China.This study aims to integrate the observational studies estimating current long-term survival of Chinese SLE patients and analyze the death-cause situation of SLE in China.The study is a systemic review of English and non-English articles using MEDLINE, EMBASE, CNKI, WANFANG, and SINOMED databases. Additional studies were found by consultation with clinical experts, browse of references in selected papers, and search of related textbooks. Our major search terms were SLE, follow-up, prognosis, survival, mortality, and China.We included cohort studies for survival analysis, and both cohort studies and case series for death-cause analysis in China.The extraction of the articles were done by 2 authors independently using predesigned charts, including characteristics of study, clinical data, analyzing data, and study quality indicators.All pooled analyses were conducted both for random-effects model and fixed-effects model. Funnel plots and Egger regression tests were applied to check potential publication bias. Heterogeneity was tested by sensitivity analysis. We identified 5 studies for survival analysis comprising 4469 Chinese patients with SLE (380 observed deaths). Thirty-six studies were suitable for death-cause analysis with 2179 observed deaths (derived from more than 20,000 Chinese patients with SLE). The overall pooled survival rates for SLE in China were 94% for 5-year survival rate and 89% for 10-year survival rate after disease onset from the year 1995 to 2013, which were similar with previous publications in Asia-Pacific area. The proportions of different causes of death showed infection (33.2%), renal involvement (18.7%), lupus encephalopathy (13.8%), and cardiovascular disease (11.5%) as the top 4 causes.The overall survival rates for Chinese patients with SLE resembled previous publications in Asia-Pacific area. But the death causes of SLE in China were of some differences indicating relatively higher proportion of infection and lupus encephalopathy and lower cardiovascular disease. Ethnicity and more aggressive treatment might have contributed to the difference in death composition.
Medicine 05/2015; 94(17):e794. DOI:10.1097/MD.0000000000000794 · 5.72 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To observe the prevalence and related risk factors of arterial stiffness measured by brachial-ankle pulse wave velocity (baPWV) in patients with systemic lupus erythematosus (SLE).
The study population included 135 patients currently enrolled in the Chinese SLE Treatment and Research group registry (CSTAR). All traditional cardiovascular risk factors and SLE-related disease factors were collected on the day of the baPWV examination.
(1)Significant differences were observed in age (P < 0.000) , family history of cardiovascular disease (P = 0.003), mean blood pressure (P = 0.000) and hemoglobin A1c (P = 0.023) between SLE patients with normal and abnormal arterial stiffness. In addition, SLE patients with abnormal arterial stiffness had lower creatinine clearance rates [85.9 (65.5-108.8) ml/min vs. 106.4 (86.8-124.6) ml/min, P = 0.011], longer disease and hydroxychloroquine duration (P = 0.002 and P = 0.022, respectively), and higher proportion of intravenous cyclophosphamide use (OR = 3.04, 95%CI:1.230-7.514, P = 0.013) as compared to patients with normal arterial stiffness. (2)After adjustment of all confounding factors, age (OR = 4.56, 95%CI: 1.863-11.133, P = 0.001), mean blood pressure (OR = 1.12, 95%CI: 1.055-1.196, P = 0.000) , disease duration (OR = 1.12, 95%CI: 1.050-1.367, P = 0.007) and the proportion of intravenous cyclophosphamide use (OR = 2.86, 95%CI: 1.364-5.979, P = 0.005) remained as independent risk factors for abnormal arterial stiffness in SLE patients.
Both traditional cardiovascular risk factors and SLE-related factors are associated with the risk of increased arterial stiffness.
Zhonghua xin xue guan bing za zhi [Chinese journal of cardiovascular diseases] 04/2015; 43(1):56-61.
[Show abstract][Hide abstract] ABSTRACT: To analyze the clinical features, laboratory tests, treatments and outcome of patients with scleroderma renal crisis (SRC).
We retrospectively reviewed the clinical and laboratory data of 16 patients with scleroderma renal crisis in Peking Union Medical College Hospital from May 2004 to May 2013. The treatment and outcome of SRC patients were also retrospectively analyzed.
There were a total of 16 SRC patients including 5 male patients and 11 females. The median age at SRC onset was (49.9 ± 12.3) years. It usually took 3.2 years from the diagnosis of systemic sclerosis (SSc) to SRC attack. Ten SRC patients belonged to diffuse cutaneous systemic sclerosis (dcSSc), and 6 patients were limited cutaneous systemic sclerosis (lcSSc). Among SRC patients, 16/16 were negative of anti-centromere antibodies (ACAs). All these 16 patients had hypertension and renal insufficiency, including 8 dialysis dependent after the onset of SRC and 7 with thrombotic microangiopathy. There were 3 patients receiving renal biopsy. The pathological findings were mainly summarized as intimal thickening and stenosis of renal arterioles. Among 13 patients with long-term followed-up, 11 patients received angiotensin converting enzyme inhibitors (ACEI), 5 patients died, 2 patients were dialysis dependent. Only 1 patient stopped dialysis after the combination treatment of ACEI and endothelin receptor antagonist. Another 5 patients didn't need dialysis.
SRC usually occurred at the early course of SSc. dcSSc was more frequent than lcSSc. ACAs were rarely found in SRC patients. The immediate and sufficient use of ACEIs was still the cornerstone of SRC treatment. Future studies are needed to evaluate the efficacy of endothelin receptor antagonist in the treatment of SRC.
Zhonghua nei ke za zhi [Chinese journal of internal medicine] 02/2015; 54(2):125-9.
[Show abstract][Hide abstract] ABSTRACT: The objective of this article is to investigate the clinical features of intestinal pseudo-obstruction (IPO) and/or ureterohydronephrosis in systemic lupus erythematosus (SLE).Sixty-one SLE patients with IPO and/or ureterohydronephrosis were analyzed retrospectively. A total of 183 cases were randomly selected as controls from 3840 SLE inpatients without IPO and ureterohydronephrosis during the same period. Patients were assigned to 1 of the 3 groups (SLE with IPO and ureterohydronephrosis, SLE with IPO, and SLE with ureterohydronephrosis). The clinical characteristics, treatments, and prognosis were compared between the 3 groups.There were 57 females and 4 males, with a mean age of 32.0 years. IPO was the initial manifestation of SLE in 49.1% of the cases, whereas ureterohydronephrosis in 32.5%. All patients were initially treated with a high-dose steroid. Thirty-one of these patients (50.8%) also received intravenous methylprednisolone pulse therapy. Two patients died of bowel perforation and lupus encephalopathy, and the other 59 patients (96.7%) achieved remission after treatment. The incidences of fever, glomerulonephritis, nervous system involvement, serositis, erythrocyte sedimentation rate elevation, hypoalbuminemia, hypocomplementemia, and anti-SSA antibody positivity were significantly higher in patients with IPO and/or ureterohydronephrosis than in the control group (without IPO and ureterohydronephrosis). Also, patients with IPO and/or ureterohydronephrosis had higher SLE Disease Activity Index scores than control patients. Compared with SLE patients with IPO, the patients with IPO and ureterohydronephrosis had a significantly higher incidence of gallbladder wall thickening, biliary tract dilatation, and serositis, whereas the patients with ureterohydronephrosis had less mucocutaneous involvement and serositis. Eight of the 47 IPO patients who initially responded well to immunotherapy relapsed; however, all responded well to retreatment with adequate immunotherapy. Of these 8 patients, 4 relapsed following poor compliance and self-discontinuation of steroid or immunosuppressant therapy. The rate of poor compliance with immunotherapy and the number of organ systems involved in patients in the recurrent IPO group were significantly higher than those in the nonrecurrent IPO group.IPO and ureterohydronephrosis are severe complications of SLE. As patients usually respond readily to early optimal steroid treatment, early diagnosis and timely initiation of glucocorticoid are important to relieve symptoms, prevent complications, and improve prognosis.
Medicine 01/2015; 94(4):e419. DOI:10.1097/MD.0000000000000419 · 5.72 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To characterize serum IgG subclass levels in several autoimmune diseases, including primary Sjogren syndrome (pSS), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and primary biliary cirrhosis (PBC). We aimed to analyze serum IgG subclass distribution and to test whether serum IgG4 levels are elevated in these diseases.Serum IgG subclass levels from 102 pSS, 102 SSc, 100 SLE, and 59 PBC patients, as well as 40 healthy controls (HCs), were measured using the immunonephelometric assay. The distribution of IgG subclasses among these autoimmune diseases was analyzed.In this cross-sectional study, serum IgG1 (IgG1/IgG) and/or IgG3 (IgG3/IgG) were significantly increased, compared with those in HCs. Only 6.34% of patients had levels of serum IgG4 >135 mg/dL. There were no significant differences in the frequency of elevated serum IgG4 levels between patients and HC. In pSS, serum IgG1 levels were much higher than those in other disease groups, whereas serum IgG2 and IgG3 levels were most prominently increased in PBC.A strikingly different serum IgG subclass distribution was detected in patients with autoimmune diseases compared with HCs. Serum IgG subclass levels also showed distinct characteristics among different autoimmune diseases. Serum IgG4 levels in these patients were lower or not much higher than those in HCs, which differed from IgG4-related diseases.
Medicine 01/2015; 94(2):e387. DOI:10.1097/MD.0000000000000387 · 5.72 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Objective
To study the factors associated with fetal loss in Chinese women with systemic lupus erythematosus (SLE) in a large cohort of SLE patients in the CSTAR (Chinese SLE Treatment and Research Group) registry.Methods
We compared the clinical characteristics and auto-antibody profiles between SLE patients with fetal loss and SLE patients with normal pregnancies. The relationship between selected variables and fetal loss was examined by univariate analysis and binary logistic regression analysis.ResultsA total of 992 patients with 2026 pregnancies were recruited. Fifty women experienced fetal loss, including 49 spontaneous abortion, eight stillbirths and three neonatal deaths. The overall fetal loss rate was 3.0% (60/2026). Arthritis and serositis were observed significantly more frequently (P < 0.05) in normal pregnancy women. The rate of thrombocytopenia was significantly increased in patients with fetal loss (30.0% vs. 16.1%, P = 0.010), while there was no statistically significant difference in the frequency of nephropathy, central nervous system involvement between the normal pregnancy group and fetal loss group. Factors that associated with fetal loss included anti-phospholipid antibodies (aPL) (OR 2.299; 95% CI 1.058–4.993; P = 0.035) and anti-Sjögren syndrome antigen A (SSA) antibody (OR 2.283; 95% CI 1.275–4.088; P = 0.005), and thrombocytopenia (OR 2.241; 95% CI 1.192–4.213; P = 0.012). However, arthritis (OR 0.544, 95% CI 0.307–0.965, P = 0.037) was associated with favorable fetal outcome.Conclusions
Both univariate analysis and binary logistic regression analysis suggest that thrombocytopenia, aPL antibodies and anti-SSA antibody are associated with fetal loss in Chinese SLE women, while arthritis may be a possible factor related to favorable pregnancy outcome.
International Journal of Rheumatic Diseases 12/2014; 18(6). DOI:10.1111/1756-185X.12542 · 1.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Objectives:
Pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality, especially in systemic sclerosis (SSc). Since there was no published study regarding PAH in the Chinese SSc population, we aimed to describe a cohort to provide some data for early diagnosis.
We evaluated 236 systemic sclerosis patients prospectively registered in the EUSTAR (European League Against Rheumatism Scleroderma Trial and Research Group) database from the Peking Union Medical College Hospital from 2009 to 2012. Among them, 33 individuals received right heart catheterisations (RHC) while the remaining patients were grouped by echocardiographic data. These patients were classified into two groups, PAH and non-PAH group. Their clinical and laboratory features were statistically analysed to identify possible risk factors for PAH in Chinese SSc population.
The possible prevalence of PAH in SSc patients was approximately 11% in our study. Digital ulcers (52.0% vs. 31.2%), telangiectasias (64.0% vs. 37.6%) and gastroesophageal reflux disease (GERD) (60.0% vs. 36.2%) were more common in SSc patients with PAH. Some laboratory results were also proved to be significantly correlated with it. Logistic regression analysis showed that telangiectasias (OR=2.888, 95% CI=1.176-7.093), presence of GERD (OR=2.592, 95% CI=1.067-6.296), anti-RNP positivity (OR=24.384, 95% CI=1.978-36.651), IgA level elevation (OR=8.745, 95% CI 4.838-122.896) and FVC/TLCO ratio (OR=97.067, 95% CI 12.475-755.271) were associated with an increased odds for PAH in SSc patients.
This study described possible predictors of PAH in Chinese SSc population, which have been supported by similar studies in other ethnic groups.
[Show abstract][Hide abstract] ABSTRACT: Background:
Systemic sclerosis (SSc) is an autoimmune disease that has three major components: inflammation, fibrosis, and vasculopathy. T-helper 17 cell (Th17) and regulatory T cell (Treg) are considered to be critical for autoimmune disease pathogenesis. The role of Th17 and Treg in SSc is still unclear. The aim of this study was to detect the presence of Th17s and CD4(+)CD25(+) Tregs in peripheral blood samples from SSc patients and to investigate the possible roles of these two T cell subsets in SSc pathogenesis.
Th17s (CD4 and IL-17 positive) and CD4(+)CD25(+) Tregs (CD4, CD25 and Foxp3 positive) in the peripheral blood mononuclear cells of 53 SSc patients and 27 healthy controls were counted by flow cytometry. The differences between SSc and control patients were analyzed. Clinical parameters, including disease duration, duration of the second symptoms, Modified Rodnan Skin Score (MRSS), anti-topoisomerase I antibody, anti-U1 ribonucleoprotein (RNP) antibody, systemic involvements, pulmonary function test (PFT) and high resolution computed tomography (HRCT) score were prospectively collected following EUSTAR (EULAR scleroderma trial and research group) protocols. The correlations between the experimental and clinical data were investigated.
The ratio of Th17 in SSc patients was significantly elevated compared to healthy controls (8.74% vs. 4.41%, P < 0.001). The amount of Th17 was positively correlated with disease duration (R = 0.531, P = 0.013) and duration of the second symptoms (R = 0.505, P = 0.023). The ratio of CD4(+)CD25(+) Treg in SSc patients also significantly differed from the healthy controls (3.04% vs. 2.24%, P = 0.018). Elevated Tregs were more frequently observed in patients with a high interstitial lung disease (ILD) score on computed tomography (24/36) compared with patients with normal ILD scores (4/12, P = 0.043). Elevated Tregs were also more often observed in patients with low carbon monoxide diffusing capacity (DLCO) (24/34) compared with patients with normal DLCO (4/11, P = 0.042).
T cell abnormalities are remarkable in systemic sclerosis. Th17s proliferate and their numbers increase with lengthened disease duration. Th17s might participate in both inflammation and fibrosis by secreting IL-17. CD4(+)CD25(+) Tregs also proliferate in SSc and may play important roles in promoting fibrosis.
Chinese medical journal 10/2014; 127(20):3557-61. DOI:10.3760/cma.j.issn.0366-6999.20141395 · 1.05 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Highlights
The present case is the first case report of PSTT associated with lupus nephritis.
The patient presented with lupus nephritis as paraneoplastic nephropathy before PSTT discovered, and rapidly achieved complete remission after hysterectomy.
This unusual ﬁndings must be interpreted appropriately to achieve the correct diagnosis.
[Show abstract][Hide abstract] ABSTRACT: Objective:
To explore the prevalence and independent influencing factors of arthritis in Chinese patients with systemic sclerosis (SSc).
A total of 248 SSc patients were enrolled in this prospective cross-sectional study in Peking Union Medical College Hospital. Demographic, clinical and laboratory data were compared in arthritis group and non-arthritis group.
Of 248 Chinese SSc patients, mean age of onset was (37.1 ± 13.7) years old and disease duration was (7.4 ± 5.2) years. Overall prevalence of arthritis was 41.1% (102/248). The presence of arthralgia(95.1% vs 15.1%), muscle weakness (38.2% vs 24.7%), short of breath (60.8% vs 45.2%), and intestinal lung disease (64.7% vs 49.3%) occurred more frequently in patients with arthritis than those without arthritis (P < 0.05). The elevation of C reactive protein, anti-Sm and anti-cyclic citrullinated peptide were more common in arthritis group than in non-arthritis group (61.4% vs 43.9%, 11.4% vs 2.5% and 28.1% vs 2/16, respectively). There were no obvious differences in the Rodnan score, digital ulcers, pulmonary arterial hypertension, and six-minute walk test between two groups (P > 0.05).
This study reveals the significant prevalence of arthritis in a large cohort of SSc patients in China. Compared to non-arthritis patients, the SSc patients with arthritis have a more severe inflammation reaction and a higher rate of intestinal lung disease.
Zhonghua nei ke za zhi [Chinese journal of internal medicine] 06/2014; 53(6):460-3.
[Show abstract][Hide abstract] ABSTRACT: Objective:
To study the clinical, cardiopulmonary functional and hemodynamic profiles of systemic sclerosis patients with pulmonary hypertension (SSc-PAH) compared with those of idiopathic pulmonary hypertension (IPAH).
Patients diagnosed with SSc-PAH or IPAH by right heart catheterization were consecutively enrolled into the study between 2011 and 2013 in Peking Union Medical College Hospital (PUMCH). Cases with pulmonary hypertension related to other diseases were excluded. Demographic data, laboratory parameters, 6 minutes walk distance (6MWD), pulmonary function and hemodynamic variables at the time of diagnosis were collected and compared between the two groups.
A total of 20 SSc-PAH patients including 19 females and one male with age of (43.1 ± 12.2) years, and 18 IPAH patients including 16 females and 2 males with age of (38.4 ± 12.4) years were enrolled in this study. Subjects in both groups had similar mean pulmonary arterial pressure, cardiac index and pulmonary vascular resistance (PVR) when recruited. Compared with IPAH patients, SSc-PAH patients showed significantly decreased all parameters including forced vital capacity (FVC)% [(77.1 ± 13.2)% vs (88.6 ± 14.9)%, P = 0.026], diffusing capacity of the lung for carbon monoxide (DLCO)% [(46.2 ± 13.1) % vs (66.6 ± 13.3)%, P < 0.001], DLCO/alveolar ventilation (VA) [(55.1 ± 14.3)% vs (75.1 ± 11.5)%, P < 0.001], and 6MWD [(365.6 ± 85.1) m vs (454.3 ± 136.8) m, P = 0.034]. In subgroup analysis of SSc-PAH patients, elevated PVR (OR 2.122, 95%CI 1.093-4.119, P = 0.026) and decreased DLCO% (OR 0.916, 95%CI 0.842-0.996, P = 0.040) were independently associated with reduced 6MWD.
Under the similar hemodynamic condition, SSc-PAH patients had more severe restrictive ventilation dysfunction and diffusion capacity dysfunction. Decreased 6MWD in SSc-PAH patients was probably related to the impairment of pulmonary function.
Zhonghua nei ke za zhi [Chinese journal of internal medicine] 05/2014; 53(5):390-3.
[Show abstract][Hide abstract] ABSTRACT: To investigate differences in genetic risk factors for rheumatoid arthritis (RA) in Han Chinese as compared with Europeans.
A genome-wide association study was conducted in China with 952 patients and 943 controls, and 32 variants were followed up in 2,132 patients and 2,553 controls. A transpopulation meta-analysis with results from a large European RA study was also performed to compare the genetic architecture across the 2 ethnic remote populations.
Three non-major histocompatibility complex (non-MHC) loci were identified at the genome-wide significance level, the effect sizes of which were larger in anti-citrullinated protein antibody (ACPA)-positive patients than in ACPA-negative patients. These included 2 novel variants, rs12617656, located in an intron of DPP4 (odds ratio [OR] 1.56, P = 1.6 × 10(-21) ), and rs12379034, located in the coding region of CDK5RAP2 (OR 1.49, P = 1.1 × 10(-16) ), as well as a variant at the known CCR6 locus, rs1854853 (OR 0.71, P = 6.5 × 10(-15) ). The analysis of ACPA-positive patients versus ACPA-negative patients revealed that rs12617656 at the DPP4 locus showed a strong interaction effect with ACPAs (P = 5.3 × 10(-18) ), and such an interaction was also observed for rs7748270 at the MHC locus (P = 5.9 × 10(-8) ). The transpopulation meta-analysis showed genome-wide overlap and enrichment in association signals across the 2 populations, as confirmed by prediction analysis.
This study has expanded the list of alleles that confer risk of RA, provided new insight into the pathogenesis of RA, and added empirical evidence to the emerging polygenic nature of complex trait variation driven by common genetic variants.
Arthritis and Rheumatology 05/2014; 66(5):1121-1132. DOI:10.1002/art.38353