Mengtao Li

Peking Union Medical College Hospital, Peping, Beijing, China

Are you Mengtao Li?

Claim your profile

Publications (46)137.56 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Objective: To analyze the epidemiology, clinical characteristics, and risk factors for systemic lupus erythematosus-related intestinal pseudo-obstruction (SLE-IPO). Methods: We retrospectively examined 85 patients with SLE with IPO as the case group and 255 randomly matched patients with SLE without any gastrointestinal manifestations as the control group, out of 4331 inpatients at the Peking Union Medical College Hospital (PUMCH) from 2003 to 2014. Results: Over the last 11 years at PUMCH, the prevalence of IPO in patients with SLE was 1.96% and the in-hospital fatality rate was 7.1%. Of these patients, 57.6% presented with IPO as the initial affected system of SLE, and the rate of misdiagnosis was about 78%. Pyeloureterectasis was the most common complication (58.9%) in patients with SLE-IPO and the incidence of biliary tract dilation was 7.1%. Patients with SLE with IPO were always diagnosed at an earlier stage of SLE with a higher frequency of hematological disturbance, polyserositis, and hypocomplementemia. Pyeloureterectasis, hypocomplementemia, and elevated C-reactive protein levels in serum were independent risk factors for IPO in SLE disease. Patients with SLE-IPO with long IPO duration and those diagnosed during late stages of SLE or concurrent with pyeloureterectasis and megacholedochus always had an unfavorable outcome. Conclusion: IPO is a rare complication, but commonly presents as the initial affected system of SLE, which can lead to a difficult diagnosis and delayed treatment. SLE-IPO occurrence concomitantly with pyeloureterectasis and megacholedochus showed a severe clinical situation in our cohort. Thus, patients with SLE-IPO with systemic smooth muscular involvement should be diagnosed early and treated aggressively.
    No preview · Article · Jan 2016 · The Journal of Rheumatology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Intracardiac thrombus (ICT) is a rare but serious complication of Behcet's disease (BD). The study was to report the clinical characteristics, imaging features, treatment, and outcomes of BD patients with ICT in a single tertiary center. A series of 626 BD patients admitted to Peking Union Medical College hospital between April 2003 and April 2013 were reviewed. A total of 12 (1.9 %) BD patients with ICT were included. ICT was presenting manifestation of BD in 5 of the 12 patients (41.7 %) and occurred mostly in men (9/12, 75 %) younger than 40 years. Patients with ICT had more arterial (66.7 vs. 7.3 %, p < 0.001) and venous involvement (75.0 vs. 8.1 %, p < 0.001) compared to those without ICT. Right heart was the most frequent site of ICT (11/12, 91.7 %). Thrombus were hyperechogenic and immobile with a broad-based attachment on echocardiography in most cases (11/12, 91.7 %). Cardiac magnetic resonance (CMR) was performed in four patients. All thrombi showed isointense on T1-weighted image, while all but one were hypointense on T2-weighted image without delayed enhancement. Remission of ICT was associated with therapy of anticoagulant, corticosteroid, and immunosuppressant in most cases (10/12, 83.3 %). There was no death, and relapses of ICT occurred in only one patient after tapering of immunosuppressant during follow-up. A diagnosis of BD should be considered when a young male patient presents with a mass in the right-sided heart accompanied by vascular lesions. Medical therapy especially adequate immunosuppressants might be the first choice for BD patients with ICT.
    No preview · Article · Jan 2016 · Clinical Rheumatology
  • [Show abstract] [Hide abstract]
    ABSTRACT: Objective: This study aims to evaluate the survival of patients with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) by a systematic review and meta-analysis. Methods: Studies were searched from MEDLINE (OVID), EMBASE, Cochrane Central Register of Controlled Trial and Scopus databases and selected according to the inclusion and exclusion criteria. Two independent reviewers extracted data from selected studies. Quality assessments were also performed using the Newcastle-Ottawa Scale. All pooled analyses were conducted both for random-effects model and fixed-effects model. Subgroup analysis and sensitivity analysis were conducted to investigate the origins of heterogeneity. Publication bias was evaluated using Begg's funnel plots and Egger's test. Results: Six studies encompassing 323 patients with SLE-associated PAH were included in the meta-analysis. The pooled 1, 3 and 5-year survival rates were 88% (95% CI 0.80-0.93), 81% (95% CI 0.67-0.90) and 68% (95% CI 0.52-0.80) respectively. No significant publication bias was shown. WHO Functional class (Fc) III/IV was found to be an independent prognostic factor of mortality. Higher mean pulmonary arterial pressure (mPAP), higher pulmonary vascular resistance (PVR), lower six minutes walking distance (6MWD), higher brain natriuretic peptide (BNP) and higher N-terminal proBNP (NT-proBNP) level were also related to poor survival. Conclusion: The long-term survival of patients with SLE-associated PAH is poor, which is worth paying greater clinical and academic attention. This study suggested that early diagnosis and management are recommended in patients with SLE-associated PAH for a better outcome of survival.
    No preview · Article · Nov 2015 · Autoimmunity reviews
  • Source
    Yiyangzi Ma · Na Shi · Mengtao Li · Fei Chen · Haitao Niu
    [Show abstract] [Hide abstract]
    ABSTRACT: Systemic autoimmune diseases are a group of heterogeneous disorders caused by both genetic and environmental factors. Although numerous causal genes have been identified by genome-wide association studies (GWAS), these susceptibility genes are correlated to a relatively low disease risk, indicating that environmental factors also play an important role in the pathogenesis of disease. The intestinal microbiome, as the main symbiotic ecosystem between the host and host-associated microorganisms, has been demonstrated to regulate the development of the body's immune system and is likely related to genetic mutations in systemic autoimmune diseases. Next-generation sequencing (NGS) technology, with high-throughput capacity and accuracy, provides a powerful tool to discover genomic mutations, abnormal transcription and intestinal microbiome identification for autoimmune diseases. In this review, we briefly outlined the applications of NGS in systemic autoimmune diseases. This review may provide a reference for future studies in the pathogenesis of systemic autoimmune diseases.
    Preview · Article · Oct 2015
  • Source
    [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.
    Preview · Article · Aug 2015 · PLoS ONE
  • Source

    Full-text · Dataset · Jul 2015
  • [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.
    No preview · Article · Jul 2015 · Clinical Rheumatology
  • [Show abstract] [Hide abstract]
    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.
    No preview · Article · Jun 2015 · Rheumatology (Oxford, England)
  • [Show abstract] [Hide abstract]
    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.
    No preview · Article · May 2015 · Arthritis and Rheumatology
  • [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.
    No preview · Article · May 2015 · Clinical and experimental rheumatology
  • [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.
    No preview · Article · May 2015 · Clinical and experimental rheumatology
  • Source
    [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.
    Full-text · Article · May 2015 · Medicine
  • [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.
    No preview · Article · Apr 2015 · Zhonghua xin xue guan bing za zhi [Chinese journal of cardiovascular diseases]
  • Mengtao Li · Xiaofeng Zeng

    No preview · Article · Apr 2015 · Zhonghua nei ke za zhi [Chinese journal of internal medicine]
  • Jiaxin Zhou · Dong Xu · Yong Hou · Qian Wang · Mengtao Li · Xiaofeng Zeng
    [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.
    No preview · Article · Feb 2015 · Zhonghua nei ke za zhi [Chinese journal of internal medicine]
  • Source
    [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.
    Preview · Article · Jan 2015 · Medicine
  • Source
    [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.
    Preview · Article · Jan 2015 · Medicine
  • [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.
    No preview · Article · Dec 2014 · International Journal of Rheumatic Diseases
  • [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. Methods: 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. Results: 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. Conclusions: This study described possible predictors of PAH in Chinese SSc population, which have been supported by similar studies in other ethnic groups.
    No preview · Article · Nov 2014 · Clinical and experimental rheumatology
  • Nan Jiang · Mengtao Li · Xiaofeng Zeng
    [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. Methods: 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. Results: 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). Conclusions: 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.
    No preview · Article · Oct 2014 · Chinese medical journal