Search

Studies on clinical features of systemic lupus erythematosus among different age-onset patients are lacking in China. This multicentre study aimed to systemically compare clinical manifestations, comorbidities, organ involvement, and laboratory findings among 797 Chinese juvenile-onset, adult-onset, and late-onset SLE (JSLE, ASLE, and LSLE) patients. They were classified into JSLE, ASLE, and LSLE groups if first diagnosed at < 18, 18–50, and > 50 years old, respectively. Chi-square test and analysis of variance were employed for categorical and continuous variables respectively. In younger-onset patients, the SLE Disease Activity Index 2000 score was significantly higher (JSLE vs. ASLE vs. LSLE = 17.43 ± 9.139 vs. 16.34 ± 8.163 vs. 14.08 ± 6.474, p = 0.031). Mucocutaneous symptoms (79.5% vs. 73.4% vs. 62.0%, p = 0.042), especially malar rash (76.1% vs. 66.1% vs. 53.5%, p = 0.011) occurred more frequently, and proteinuria rate was higher (54.5% vs. 56.3% vs. 36.6%, p = 0.007). In later-onset patients, cardiopulmonary involvement increased (11.4% vs. 24.3% vs. 29.6%, p = 0.012). In ASLE, hypoalbuminemia rate elevated (46.6% vs. 59.9% vs. 47.9%, p = 0.015). Our study demonstrated in a Chinese population that JSLE may be more active and suffer mucocutaneous disorders, while LSLE tended to suffer cardiopulmonary involvement at-onset. These findings may help identify treatment priorities when facing different age-onset SLE patients.

Environmental exposures interact with genetic factors has been thought to influence susceptibility of systemic lupus erythematosus (SLE) development. To evaluate the effects of environmental exposures on SLE, we conducted a population-based cohort study across Jiangsu Province, China, to examine the associations between the living environment including air and water pollution, population density, economic income level, etc. and the prevalence and mortality of hospitalized SLE (h-SLE) patients. A total of 2231 h-SLE patients were retrieved from a longitudinal SLE database collected by the Jiangsu Lupus Collaborative Group from 1999 to 2009. The results showed that: It existed regional differences on the prevalence of h-SLE patients in 96 administrative districts; The distribution of NO2 air concentration monitored by atmospheric remote sensors showed that three of the ultra-high-prevalence districts were located in the concentrated chemical industry emission area; h-SLE patient prevalence was positively correlated with the excessive levels of nitrogen in drinking water; The positive ratio of pericarditis and proteinuria was positively correlated with the prevalence of h-SLE patients and pollution not only induced a high h-SLE patient prevalence but also a higher mortality rate, which might be attributed to NOx pollution in the air and drinking water. In summary, our data suggested that NOx in air and drinking water may be one of the important predispositions of SLE, especially for patients with renal involvement.

Objective To evaluate the association and dose–response pattern between antimalarial drugs and overall and cause specific mortality in SLE patients. Methods Medical records including information on HCQ/chloroquine (CQ) prescription were extracted from Jiangsu Lupus database. The database was designed to collect data from SLE patients that first-hospitalized during 1999–2009 in Jiangsu province, China, and a follow-up for survival status was performed in 2010 and 2015. Cox and restricted cubic spline models were used to estimate the hazard ratio and 95% CI. Results We identified 221 deaths among 2446 SLE patients in total. Compared with non-users, decreased overall mortality was associated with either HCQ or CQ users, with adjusted hazard ratio (95% CI) of 0.49 (0.35, 0.67) and 0.49 (0.27, 0.87), respectively. The association between HCQ/CQ and overall mortality was similar across subgroups, such as patients with comorbidities and organ involvements. Interestingly, both the time and the daily dosage of HCQ/CQ use were related to decreased mortality of SLE in a linear dose–response relationship. In cause specific analyses, HCQ/CQ was inversely associated with death from renal insufficiency and other organ (cardiopulmonary, gastrointestinal and haematological) involvements, with adjusted hazard ratio (95% CI) of 0.23 (0.09, 0.55) and 0.25 (0.10, 0.62), respectively, yet it was not significantly associated with mortality from infection and neuropsychiatric involvements. Conclusion Antimalarial drugs were associated with lower risk of SLE mortality, especially renal insufficiency- and other organ involvement-related death. The protective effects for survival might be augmented by adherence and full dosage of these drugs.

The objective of the study was to investigate the long-term treatment effects and predictive factors for treatment response and disease relapse for Takayasu's arteritis (TA). Eighty-one patients were recruited from the Department of Rheumatology, Zhongshan Hospital, Fudan University, between January 2009 and January 2015. The follow-up duration ranged from 6 to 36 months. Patients were divided into three groups: clinical remission (CR; n = 59); treatment-resistant (TR; n = 11); and disease relapse (DR; n = 11). Signs/symptoms, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and imaging items were recorded at baseline and at each visit. Kerr's criteria, physician's global assessment, and Indian Takayasu Clinical Activity Score (ITAS2010) were used to evaluate disease activity. Incipient disease was more common in CR patients compared with DR cases (69.49 vs. 36.36%, p = 0.05). Fewer patients aged 40 years were in the CR group in comparison with the DR group (57.63 vs. 90.91%, p = 0.04). In TR patients, high CRP levels (63.74 vs. 23.73%, p = 0.01) and aortic arch involvement (70.00 vs. 24.14%, p 0.01) were more common in comparison with CR cases. Patients with high CRP levels ( 25 mg/L) (OR = 1.61, p = 0.03) carried a higher risk for treatment resistance. Age 40 years (OR = - 2.82, p = 0.03), incipient disease (OR = - 2.47, p = 0.01), and treatment with cyclophosphamide (OR = - 2.07, p = 0.03) and hydroxychloroquine (OR = - 1.91, p = 0.05) could prevent disease relapse. Patients with high CRP levels carry a high risk of treatment resistance. In patients with incipient disease, aged 40 years, treatment with cyclophosphamide and hydroxychloroquine protects against disease relapse.

Iguratimod (IGU) is a novel disease-modifying anti-rheumatic drug (DMARD) in rheumatoid arthritis (RA). Like other DMARDs, IGU exhibited significant differences in effectiveness and safety. Aim: The aim of this study was to identify genetic predictorsof efficacyand toxicity of IGU in patients with RA. Materials & methods: Seven SNPs from IGU-metabolizing genes were genotyped in 272 IGU-treated patients with RA. Results: ABCG2 rs2231142 A allele conferred a higher response to IGU, while NAT2 rs1495742 G carriersconferred a lower response to IGU. CYP2C19*2 rs4244285 A carriers had higher risk for IGU-induced toxicity compared to the GG carriers. Conclusion: Our study suggests that the polymorphisms of ABCG2 (rs2231142), NAT2 (rs1495741)and CYP2C19*2 (rs4244285) may help to predict thetherapeutic effectiveness and toxicity of IGU in patients with RA.

The objective of this study was to systemically and comprehensively evaluate the associations between smoking and disease outcomes in patients with ankylosing spondylitis (AS). Information on smoking, clinical features, and sociodemographic characteristics was collected by a questionnaire administered directly to the patient. Group differences were analyzed by t test or chi-square test. Logistic regression analysis was conducted with the Bath AS Disease Activity Index (BASDAI), Bath AS Functional Index (BASFI), C-reactive protein, and erythrocyte sedimentation rate as the dependent variables and different stratification of smoking duration, smoking intensity, and cumulative smoking as independent variables. In order to compare our results with previous studies, meta-analysis was performed to calculate standardized mean difference (SMD) for relationship between outcomes and smoking status. A total of 1178 AS patients were analyzed. Compared with non-smokers, the risk of having active disease (BASDAI ≥ 4) was higher in patients who smoked at least 15 years, or 15 cigarettes per day, or 15 pack-years (OR = 1.70 [1.06, 2.73], 1.75 [1.08, 2.82], and 1.97 [1.06, 3.67], respectively); and smokers had increasing risk of BASDAI ≥ 4 with increasing years of smoking, or cigarettes per day, or pack-years (p-trend = 0.010, 0.008 and 0.006, respectively). The risk of having active disease was higher in patients who smoked at least 15 cigarettes per day or 15 pack-years (OR = 1.74 [1.06, 2.84] and 2.89 [1.56, 5.35], respectively), with increasing number of cigarettes per day and pack-years. Smokers had an increased risk of BASFI ≥ 4 (p-trend = 0.040 and 0.007, respectively). By meta-analysis, current, former and ever smokers had significantly higher BASDAI (SMD = 0.34 [0.18, 0.48], 0.10 [0.01, 0.19], and 0.27 [0.20, 0.34], respectively) and BASFI (SMD = 0.35 [0.16, 0.55], 0.30 [0.22, 0.39], and 0.35 [0.21, 0.50], respectively) compared to non-smokers. Smoking is a risk factor for greater disease activity and worse functioning in AS patients.

To assess the effectiveness of cyclophosphamide (CYC) versus methotrexate (MTX) for active Takayasu's arteritis (TA). The current study was based on a cohort of TA at Zhongshan Hospital, Fudan University. TA was diagnosed using the 1990 American College of Rheumatology criteria. Fifty-eight subjects receiving induction treatment with CYC (n = 46) or MTX (N = 12) were included in the analysis. Effectiveness and toxicity were assessed in all 58 cases. Clinical remission was defined as: Kerr score reduction to ≤ 1 and glucocorticoids (GC) treatment at a dose of ≤ 0.2 mg/kg/day (≤ 15 mg/day) at the end of the 6th month. At the baseline, the CYC group had higher Kerr scores (60.9% vs. 16.7% at ≥3, p = 0.044), higher ESR (55 ± 52 vs. 25 ± 22 mm/H, p = 0.048), ITAS_ESR (12.4 ± 1.7 vs. 9.1 ± 1.1 mg/L, p = 0.043). The 6-month clinical remission rate was 71.7% vs. 75% in the CYC and MTX group, respectively. In the CYC group, a significant decrease was observed in ESR (55 ± 52 vs. 25 ± 48 mm/H, p = 0.008), hs-CRP (27 ± 23 vs. 6.9 ± 6.6 mg/L, p = 0.007), ITAS (11.7 ± 2.2 vs. 7.0 ± 1.5, p = 0.048), and ITAS_ESR (7.1 ± 2.0 vs. 12.4 ± 1.7, p = 0.033). However, no significant reductions in these measures were demonstrated in the MTX group. Whole-body contrast enhanced magnetic resonance angiography (MRA) revealed significant radiologic improvement (wall enhancement scores: 4.2 ± 2.3 vs. 10.3 ± 3.8, p = 0.032) in the CYC group, but not in the MTX group. No severe adverse events occurred in any subject. Cyclophosphamide could be a better choice than methotrexate as induction treatment for patients with more severe Takayasu's arteritis.

The study aimed to determine whether unique clinical patterns of AS may exist in China, specifically to explore the different clinical manifestations caused by gender, HLA-B27 status, and age at disease onset. The multicenter cross-sectional survey was conducted and 1251 patients were enrolled across China, representing a broad spectrum of Chinese AS patients. The mean age at onset and diagnosis were 29.2 (11.4) and 33.5 (12.6) years, respectively. The male/female ratio was 2.7:1. Acute anterior uveitis (AAU) was experienced in 10.3% of AS patients and 9.1% patients had juvenile-onset AS (JoAS). Men were significantly younger at onset and diagnosis and showed a higher frequency of HLA-B27 positivity, JoAS, and AAU than women. HLA-B27-positive patients had a younger age of onset than HLA-B27-negative patients. HLA-B27-positive patients were nearly three times as likely to develop AAU than negative patients (P = 0.04). JoAS patients had a family history of AS more often than adult-onset AS (AoAS) patients, and 4.9% of JoAS patients underwent surgical treatments, a rate more than six times that of AoAS patients (P = 0.01). Men had higher levels of C-reactive protein than women, as did HLA-B27 positives compared to negative patients, and JoAS compared to AoAS (all P

Thalidomide is effective for treating severe cutaneous lupus patients. The aim of this study was to observe the optimum effective and maintenance doses of thalidomide to maximize clinical benefit and minimize side effects for patients with cutaneous lupus in China. Sixty-nine patients with lupus rash from eight hospitals in China were enrolled and treated with different doses of thalidomide. We started the dose of thalidomide at 25 mg daily and gradually increased administration dose once a week until erythema was markedly improved. The effective and maintenance doses were documented. The size of skin lesions was noted once a week. Systemic lupus erythematosus disease activity index (SLEDAI) score, levels of erythrocyte sedimentation rate (ESR), and serum TNF-α were measured before and after treatment. The remission rates were evaluated weekly until 8 weeks. Sixty-eight percent of patients showed an effective dose of 50 mg daily; another 13, 10, and 9 % of patients had an effective dose of 100, 75, and 25 mg daily, respectively. The maintenance dose was 50 mg daily for 71 % of the patients, and 100, 75, and 25 mg daily for 9, 14, and 6 % of the patients. SLEDAI score and serum ESR levels significantly decreased 4 weeks after thalidomide treatment. At the end of the fourth week, the rates of complete remission, partial remission, and no response were 56 % (n = 39), 41 % (n = 28), and 3 % (n = 2). At the eighth week, the rate of total remission rose up to 100 %. The most common side effects were drowsiness and constipation. No peripheral neuropathy was observed in these patients. Thalidomide at a dose of 50 mg daily may offer a better benefit to risk ratio in the treatment of Chinese cutaneous lupus patients.

Objective.Left atrial function plays a key role in maintaining an optimal cardiac output. Left ventricular diastolic dysfunction has been reported in systemic lupus erythematosus (SLE), but its effect on left atrial function has been largely overlooked. Our aim was to assess left atrial performance using real-time 3-D echocardiography (RT3DE) technology in patients with SLE.Methods.Our study included 102 patients with SLE without any cardiac symptoms, and 32 healthy controls. According to the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), all subjects were classified into 3 groups: healthy controls, patients with an SDI = 0, and patients with an SDI ≥ 1.Results.Left atrial volume indexed to body surface area was dilated in subjects with SLE, whereas the left atrial passive emptying fraction (EF) was lower. Left atrial active EF was significantly higher in the SDI = 0 group than in controls (46.4 ± 9.1% vs 30.0 ± 10.3%, p < 0.05); however, it was significantly lower in the SDI ≥ 1 group than in the SDI = 0 group (41.2 ± 9.8% vs 46.4 ± 9.1%, p < 0.05). By multivariate linear analysis, the SDI was independently and positively associated with left atrial volume index and inversely associated with left atrial total function.Conclusion.Our study demonstrated that left atrial mechanical function and volume are impaired in SLE, particularly in patients with an SDI ≥ 1 and disease activity. RT3DE may have better diagnostic value than traditional echo indexes in detecting subclinical cardiac dysfunction in patients with SLE.

Bone damage around the joints is one of the major pathophysiological mechanisms that leads to rheumatoid arthritis (RA) chronic disability. Serum tartrate-resistant acid phosphatase 5b (TRACP-5b) is secreted by osteoclasts, its activity can be used as a clinically relevant bone resorption marker. The aim of this study was to test whether the measurement of serum levels of TRACP-5b in patients with RA would correlate with measures of disease activity and with responses to therapy.Fifty-six patients were randomly assigned to receive recombinant human cytotoxic tlymphocyte-associated antigen-4 immunoglobulin (RhCTLA4-Ig), infliximab or methotrexate (MTX). The clinical and serologic indicators of RA activity were evaluated at baseline and at 24 weeks. Serum TRACP-5b was measured by Enzyme-linked Immunosorbent Assay (ELISA) at 0, 12 and 24 weeks. Hand X-rays were obtained at baseline.At baseline, the levels of TRACP-5b correlated with the severity of X-ray damage, disease duration (r = 0.332, P = 0.012), and tender joint count (r = 0.408, P = 0.002). The 24 weeks values of TRACP-5b for RhCTLA4-Ig group and infliximab group differed significantly from the baseline values in each group (P0.05; P0.05), whereas only the value for RhCTLA4-Ig group differed significantly from the 24 weeks value for the MTX group (P0.01). Considering the two biologics-treated groups together, the TRACP-5b levels at 24 weeks differed significantly from the baseline values only in those patients who reached an ACR70 level (P0.05).Measurement of serum TRACP-5b in RA patients reflects clinical and radiological measures of disease activity, treatment with certain biologics, and degree of response to therapy. TRACP-5b should be investigated further as a potential biomarker to predict response to therapy, including slowing of radiographic progression.

To explore the clinical features and prognosis of patients with lupus nephritis (LN) in a large multicenter lupus cohort of Jiangsu Province.Medical records of 2 078 systemic lupus erythematosus (SLE) inpatients from 15 hospitals at the first admission from 1999 to 2012 were reviewed and classified into two groups with LN or without. The clinical features between two groups were compared with Mann-Whitney U or Chi-square test and potentially associated factors tested by Cox regression.A total of 883 (42.5%) hospitalized lupus patients were diagnosed as LN. And the median age at disease onset of LN patients was lower than that of those without LN [(30 ± 11) vs (32 ± 12) years, P0.01]. Cardiopulmonary involvement, neuropsychiatric disorder, gastrointestinal dysfunction, hematologic disease, ophthalmopathy, SLEDAI score9 at admission and SLE disease activity index (SLEDAI) score9 at discharge were more often seen in patients with LN compared to those without LN (31.5%, 7.9%, 13.9%, 69.0%, 1.5%, 77.4%, 29.8% vs 18.8%, 5.1%, 6.8%, 63.1%, 0.3%, 43.1%, 8.1%, all P0.01). The mortality rates at 1 or 5 years after first admission were both significantly higher in LN patients than those without LN (7.2%, 15.0% vs 3.1%, 6.3%, P0.01). Independent predictors for mortality in patients with LN were neuropsychiatric involvement[hazard ratio (HR) 2.46], SLEDAI score9 at discharge (HR 2.34), increased serum creatinine (HR 2.21) and elevated alanine aminotransferase and (or) aspartate transaminase (HR 2.09) whereas glucocorticosteroid therapy (HR 0.18) was a protective factor.LN is one common complication of SLE patients during an early stage. And LN patients are more prone to present other vital organ involvement, higher disease activity and worse treatment outcomes. When accompanied with neuropsychiatric involvement, increased serum creatinine or elevated transaminase, worse prognosis is expected. Glucocorticosteroid treatment may offer some benefits.

Objective To identify early signs associated with poor prognosis in Chinese patients with systemic lupus erythematosus (SLE) through a large population-based follow-up study. Methods Medical records of > 2,500 SLE patients that first hospitalized between 1999–2009 were collected from 26 centers across Jiangsu province, China, and entered into a database. These patients were followed-up for 5 to 15 years, and those remained contact and had known survival status in 2015 were assessed for the association of factors presented at the initial hospitalization with mortality at two time points (≤1year and > 1year). The independency of mortality factors was evaluated using multivariate Cox regression analysis. Results Among 1,372 patients we assessed, 92.3% were women and 17.2% were deceased in 2015. The main causes of death were infection (30.1%), neuropsychiatric impairment (14.8%), renal failure (14.4%) and cardiopulmonary involvement (8.5%). Hazard ratios (HR) of independent predictors for mortality (≤1year and > 1year, respectively) included hospital presentation of neuropsychiatric involvement (2.03 and 1.91), cardiopulmonary involvement (1.94 and 1.61) and increased serum creatinine (2.52 and 2.58). Patients older than 45 years and with disease durations more than 2 years at admission had unfavorable short-term outcome (HR 1.76 and 1.79), while the presence of anti-dsDNA and anti-Sm antibodies indicated diverse prognosis after 1 year (HR 1.60 and 0.45). Treatment with cyclophosphamide was beneficial for patient’s first-year outcome (HR 0.50), and anti-malarial drugs significantly reduced the risk of mortality over different time points (HR 0.48 and 0.54). SLEDAI score, proteinuria or hypocomplementemia was not independently associated with the outcome in this cohort. Conclusion SLE patients presented with vital organ damages rather than active disease at initial hospitalization are likely to have a poor outcome, especially for those with neuropsychiatric, cardiopulmonary involvements and renal insufficiency. Early and effective intervention with the use of anti-malarial drugs may decrease mortality.

Objective.To investigate the mortality of hospitalized patients with systemic lupus erythematosus (SLE) and determine the influential factors associated with poor prognosis.Methods.Medical records of 1956 SLE inpatients from 15 hospitals during the period January 1, 1999, to December 31, 2009, were reviewed. All patients were followed up in January 2010. Potential factors associated with mortality were analyzed, comparing patients who were living with those who were deceased. The independency of those factors significantly related to death was determined by Cox regression analysis.Results.Male to female ratio was 1:15 in this cohort; median age at disease onset was 30 years. Hematologic (70.0%), mucocutaneous (68.2%), musculoskeletal (57.9%), and renal (48.7%) involvements were most often seen in these patients at time of admission. The overall mortality was 8.5% (n = 166), with infection (25.9%), renal failure (19.3%), and neuropsychiatric lupus (18.7%) the leading 3 causes of death. Independent predictors for mortality in this cohort of SLE patients were neuropsychiatric involvement [hazard ratio (HR) 2.19], anemia (HR 1.69), SLEDAI score > 8 at discharge (HR 1.64), increased serum creatinine (HR 1.57), low serum albumin (HR 1.56), cardiopulmonary involvement (HR 1.55), and patient untreated before admission (HR 1.48), whereas the use of antimalarial drugs (HR 0.62) and positive anti-Sm antibody (HR 0.60) were shown to be protective factors.Conclusion.SLE patients with delayed treatment and refractory disease have poorer prognosis. A high incidence of death would be expected if they have neuropsychiatric involvement, anemia, azotemia, or cardiopulmonary involvement. Combination therapy with antimalarial drugs may provide some benefit to patients with SLE.