Mengtao Li

Chinese Academy of Medical Sciences, Peping, Beijing, China

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Publications (40)118.78 Total impact

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    ABSTRACT: To characterize the clinical features of IgG4-related disease (IgG4-RD) in China. A prospective cohort study of IgG4-RD was carried out in Peking Union Medical College Hospital between 2011 and 2013. Patients with newly diagnosed IgG4-RD were enrolled. A total of 118 patients with IgG4-RD were enrolled, including 82 males and 36 females, aged 53.1 (s.d. 13.6) years. The most common symptom at onset was lacrimal gland swelling (38/32.2%). A range of organs were involved: 77 patients (65.3%) had lymphadenopathy, 76 (64.4%) had sialadenitis, 60 (50.8%) had dacryoadenitis, 45 (38.1%) had autoimmune pancreatitis, 32 (27.1%) had pulmonary involvement, 31 (26.3%) had periaortitis/retroperitoneal fibrosis, 29 (35.4% of male patients) had prostatitis and 29 (24.6%) had renal involvement. In addition, there were 21 (17.8%) cases of sclerosing cholangitis, 15 (12.7%) of sinusitis and 10 (8.5%) of inflammatory pseudotumour. Uncommon manifestations included mediastinal fibrosis, skin involvement, sclerosing thyroiditis, hypophysitis, orchitis and colitis. Multiple organ involvement was observed in 93 patients, whereas only 4.2% had only a single organ involved. A history of allergy was reported in 73 (61.9%) patients. The serum IgG4 level was elevated in 97.5% and was correlated with the number of organs involved. Most patients were treated with glucocorticoids alone or in combination with immunosuppressive drugs, and the majority usually improved within 3 months. IgG4-RD is a systemic inflammatory and sclerosing disease. Parotid and lacrimal involvement (formerly called Mikulicz's disease), lymphadenopathy and pancreatitis are the most common manifestations. Patients with IgG4-RD showed favourable responses to treatment with glucocorticoids and immunosuppressive agents. © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.
    Rheumatology (Oxford, England) 06/2015; DOI:10.1093/rheumatology/kev203 · 4.44 Impact Factor
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    ABSTRACT: Objective To investigate autoantibody against endothelin-1 receptor type A (ETRA-Ab) in patients with systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-PAH). Its possible autoantibody-mediated pathogenesis in the development of SLE-PAH has also been explored. Methods ETRA-Ab in serum of SLE-PAH and controls were detected by establishing a human ETRA epitope peptide-based ELISA. An exploratory cohort of SLE-PAH (n=76) and an independent validation cohort with right heart catheterization confirmed SLE-PAH (n=82) were enrolled. The clinical relevance of ETRA-Ab in SLE-PAH was analyzed. Proliferation of vascular smooth muscle cells (SMCs) and permeability of endothelial cells (ECs) in vitro under the stimulation of polyclonal ETRA-Ab IgG were assessed. The expressions of PAH-related markers, i.e., 5-HTT, PDGFR-b, VEGF-A and PDGF-B were measured by qPCR. The effect of ETRA-Ab in vivo was also determined in a suboptimal-dose monocrotaline-induced PAH model with the assessment of right ventricle hypertrophy index, pulmonary angiography and pathology parameters. Results ETRA-Abs was presented with a higher occurrence in SLE-PAH (41.5∼42.1%) compared with controls (0∼17.1%). There was a significant correlation between the titer of ETRA-Ab and pulmonary arterial systolic pressure estimated by echocardiography (r=0.2978, p=0.0038) or measured by right heart catheterization (r=0.2159, p=0.0257) in SLE-PAH. ETRA-Ab can promote SMCs proliferation, disrupt endothelial barrier and up-regulate PAH-related markers expression, which can be blocked in the presence of ETR antagonist. ETRA-Ab aggravated right ventricle hypertrophy and vascular remodeling in vivo. Conclusions This study identified ETRA-Ab in SLE, as a new biomarker of mechanistic relevance, which may mediate PAH development in SLE. This article is protected by copyright. All rights reserved. © 2015, American College of Rheumatology.
    05/2015; DOI:10.1002/art.39212
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    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; · 2.97 Impact Factor
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    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.
    Clinical and experimental rheumatology 05/2015; · 2.97 Impact Factor
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    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 · 4.87 Impact Factor
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    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.
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    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] 01/2015; 43(1):56-61.
  • Mengtao Li, Xiaofeng Zeng
    Zhonghua nei ke za zhi [Chinese journal of internal medicine] 01/2015; 54(1):6-8.
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    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 · 4.87 Impact Factor
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    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 · 4.87 Impact Factor
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    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; DOI:10.1111/1756-185X.12542 · 1.77 Impact Factor
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    ABSTRACT: 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.
    Clinical and experimental rheumatology 11/2014; 32 Suppl 86(6):115-21. · 2.97 Impact Factor
  • Nan Jiang, Mengtao Li, Xiaofeng Zeng
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    ABSTRACT: 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.
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    ABSTRACT: •An improved YASSO model is proposed by considering residual spatial autocorrelation.•Model prediction errors are minimised using GIS data at the appropriate spatial scale.•Topographical factors describe 24-49% of variation in soil carbon.
    08/2014; 9:26-8. DOI:10.1016/j.gynor.2014.05.003
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    ABSTRACT: To explore the prevalence and independent influencing factors of arthritis in Chinese patients with systemic sclerosis (SSc).
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    ABSTRACT: 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).
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    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.
    05/2014; 66(5):1121-1132. DOI:10.1002/art.38353
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    ABSTRACT: We investigated the characteristics of Chinese SLE patients by analyzing the association between specific autoantibodies and clinical manifestations of 2104 SLE patients from registry data of CSTAR cohort. Significant (P < 0.05) associations were found between anti-Sm antibody, anti-rRNP antibody, and malar rash; between anti-RNP antibody, anti-SSA antibody, and pulmonary arterial hypertension (PAH); between anti-SSB antibody and hematologic involvement; and between anti-dsDNA antibody and nephropathy. APL antibody was associated with hematologic involvement, interstitial lung disease, and a lower prevalence of oral ulcerations (P < 0.05). Associations were also found between anti-dsDNA antibody and a lower prevalence of photosensitivity, and between anti-SSA antibody and a lower prevalence of nephropathy (P < 0.05). Most of these findings were consistent with other studies in the literature but this study is the first report on the association between anti-SSA and a lower prevalence of nephropathy. The correlations of specific autoantibodies and clinical manifestations could provide clues for physicians to predict organ damages in SLE patients. We suggest that a thorough screening of autoantibodies should be carried out when the diagnosis of SLE is established, and repeated echocardiography annually in SLE patients with anti-RNP or anti-SSA antibody should be performed.
    Research Journal of Immunology 04/2014; 2014:809389. DOI:10.1155/2014/809389
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    ABSTRACT: Background: No previous study has been done on whether systemic lupus erythematosus (SLE) disease activity is related to the hemodynamics and right ventricular (RV) function in patients with SLE-associated pulmonary artery hypertension (SLE-APAH). Methods and Results: This study prospectively recruited 54 patients (mean age, 32.8±8.4 years; 92.6% female) with SLE-APAH, including 34 patients with SLE disease activity index (SLEDAI) <5 (low score) and 20 with SLEDAI ≥5 (high score). All patients underwent right heart catheterization and iloprost inhalation, and echocardiography was performed before and immediately after iloprost inhalation. There was no difference in baseline mean pulmonary artery pressure (mPAP) between the 2 groups; pulmonary vascular resistance (PVR) was significantly higher and cardiac index was significantly lower in the low-SLEDAI group. The patients with low SLEDAI had larger RV size and worse RV systolic function on echocardiography. After iloprost inhalation, the patients with low SLEDAI had a greater decrease in mPAP and PVR than those with high SLEDAI, while significantly increased RV systolic function was found only in the low-SLEDAI group. Conclusions: SLE activity is related to hemodynamics and RV function in SLE-APAH patients, and those with low SLEDAI might have better acute response to vasodilator inhalation.
    Circulation Journal 02/2014; 78(5). DOI:10.1253/circj.CJ-13-1280 · 3.69 Impact Factor
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    ABSTRACT: Behçet's disease (BD) is a rare, chronic, relapsing, systemic, immune-mediated vasculitis and the etiology remains to be defined. This study investigated single-nucleotide polymorphisms (SNP) of tyrosine-protein phosphatase non-receptor type 2 (PTPN2) and inducible T-cell co-stimulator-ligand gene (ICOSLG) in Chinese Han BD patients and healthy controls because SNPs of these two genes are associated with risk of developing other auto-inflammation diseases. A total of 407 BD patients and 679 ethnically matched healthy controls were recruited for genotyping of PTPN2 rs1893217, rs2542151, rs2847297 and rs7234029 SNPs and ICOSLG rs2838519 and rs762421 SNPs using a Sequenom MassArray system. PTPN2 rs1893217 was associated with risk of developing BD (χ2=10.01, pc=0.040), while the PTPN2 rs2542151 genotype had a weak association in basic genotype analysis (χ2=7.49, p=0.024), but it could not withstand the strongest Bonferroni correction (pc=0.14). In contrast, PTPN2 rs2847297 and rs7234029 and ICOSLG rs2838519 and rs762421 did not correlate with BD risk. Moreover, logistic analysis with the additive, dominant and recessive genetic models did not reveal any statistical difference between BD cases and controls (pc>0.05). In addition, associations were observed between the two SNPs (rs1893217, rs2542151) and the patients with gastrointestinal involvement (pc=0.027, pc=0.032, respectively). PTPN2 variant rs1893217 was associated with risk of BD development in a Han Chinese population. Further study will confirm this finding and investigate the role of PTPN2 in development of BD.
    Clinical and experimental rheumatology 01/2014; · 2.97 Impact Factor