The Chinese systemic lupus erythematosus (SLE) treatment and research group (CSTAR) provides major clinical characteristics of SLE in China and establishes a platform to provide resources for future basic and clinical studies. CSTAR originated as a multicentre, consecutive, and prospective design. The data were collected online from 104 rheumatology centers, which covered 30 provinces in China. The registered patients were required to meet four or more of the American College of Rheumatology (ACR) criteria for the classification of SLE. All CSTAR centers use the same protocol-directed methods to provide uniform evaluations, which included demographic data, clinical features, laboratory examinations, and disease activity evaluations. The patient samples, including DNA samples and sera, were also collected for further quality controls and additional studies. Preliminary analysis from 2104 baseline evaluations was available for this analysis. Of 1914 female and 190 male patients (F:M=10.1), the mean age at onset was 29.2 y with confirmed diagnosis one year later at the age of 30.3 y. Eighty four (4.2%) of 2002 patients had a family history of rheumatic diseases, including 34 (1.7%) cases with SLE. In addition, one hundred and seven (5.2%) abnormal pregnancies were recorded among 2026 experiences. The characteristics of the CSTAR cohort were compared to similarly sized cohorts from other studies. We found that 56.1% of patients presented with concurrent hematological disorders compared to only 18.2% of European patients. Moreover, 47.4% of patients presented with nephropathy compared to 27.9% of European patients. Conversely, neurological manifestations were only seen in 4.8% of Chinese SLE patients compared to 19.4% of European patients, 12.1% of U.S. patients, 22.8% of Malaysian patients and 26.4% of Latin Americans. Pulmonary arterial hypertension and interstitial lung diseases were complications identified in 3.8% and 4.2% of Chinese lupus patients, respectively. The CSTAR registry has provided epidemiological data and phenotypes of Chinese patients with SLE, and has demonstrated several differences between ethnicities. Clinical data and biologic samples would be valuable resources for future translational studies with national and international collaboration.

No abstract

Peking Union Medical College Hospital (PUMCH) has started a single-center right heart catheterization (RHC)-based pulmonary arterial hypertension (PAH) study in systemic lupus erythematosus (SLE) since 2006. The baseline characteristics of these patients were described and the risk factor for PAH in lupus was identified.The demographic, clinical, laboratory, and treatment characteristics of SLE patients with PAH when they were registered were collected as the baseline data. A case-control study was conducted by taking the admitted SLE-non-PAH patients adjusted for age and gender in a 4:1 ratio during the same period as the controls. The associated variables were examined by binary multivariate logistic regression analysis to identify possible risk factors. A total of 111 RHC-confirmed SLE-PAH patients were enrolled, with the onset age of 34.6 ± 8.6 years old and the average SLE duration of 5 years. RHC revealed mPAP as 46.4 ± 11.4 mm Hg, CI as 2.7 ± 0.8 L/min × m2, and PVR as 10.5 ± 4.8 WU. 46% of patients were WHO Fc I–II. All patients were treated with immunosuppressive agents and 65% patients had PAH-targeted therapy. The case-control study had confirmed 2 independent risk factors previously published: pericardial effusion (OR = 21.290, P < 0.001) and anti-RNP antibody (OR = 12.399, P < 0.001). Meanwhile, 6 independent variables were discovered: baseline SLE duration (OR = 1.118, P = 0.007), interstitial lung disease (OR = 17.027, P < 0.001=, without acute rash (OR = 3.258, P = 0.019), anti-SSA antibody (OR = 4.836, P = 0.004), SLEDAI≤9 (OR = 26.426, P < 0.001), ESR≤20 mm/h (OR = 12.068, P < 0.001), and uric acid > 357 μmol/L (OR = 9.666, P < 0.001) to be associated with PAH in SLE patients.The PUMCH study has shown that SLE patients complicated with PAH are usually earlier diagnosed and have less disease severity than patients without PAH. The immunosuppressive therapy rate and the PAH target therapy rate were high, which is consistent with reports from Western countries. This study has confirmed that pericardial effusion and positive anti-RNP antibody are risk factors for SLE-associated PAH. Long SLE disease duration, the presence of interstitial lung disease, without acute skin rash, positive anti-SSA antibody, low SLEDAI and ESR, and high uric acid levels are also associated with PAH in SLE patients.

The possible prevalence of PAH was 3.8% in Chinese patients with SLE in the CSTAR registry. The significant association of pericarditis, pleuritis and anti-RNP positivity with PAH suggests that higher disease activity and vasculopathy may both contribute to the development of PAH in SLE, which need be treated aggressively to improve prognosis.

No abstract

BackgroundWe aimed to investigate the clinical characteristics of 411 Chinese Takayasu’s arteritis (TAK) patients using a retrospective analysis.MethodsWe retrospectively reviewed 810 medical charts of patients with a diagnosis of TAK who were admitted to Peking Union Medical College Hospital from 1990 to 2014. 411 patients with a complete dataset were finally included in the analysis. The demographic data, clinical features, angiographic patterns, and TAK-related surgical procedures were collected and analyzed.ResultsThe median age at disease onset was 23 (18, 30) years old, with a median disease duration of 21 (6, 60) months; 325 (79.1%) were female. The angiographic involvement pattern was type I in 91 (22.1%) patients, type IIa in 16 (3.9%) patients, type IIb in 16 (3.9%) patients, type III in 12 (2.9%) patients, type IV in 26 (6.3%) patients, and type V in 250 (60.8%) patients. Subclavian arteries (79.8%) were the most commonly involved, followed by carotid arteries (79.1%). The occurrence rate (4.1%) of aortic aneurysm in this study was low; 119 operations and interventions were performed. The most common cause of death in this study was heart failure.ConclusionSubclavian arteries, carotid arteries, and type V were the most frequently involved arteries and angiography pattern in this Chinese TAK study. The difference in angiographic features may lead to differences in clinical manifestations. Surgical operation and interventions should be performed at different stages of the disease course.

Objective This study aims to exhibit the prognosis, both mortality and morbidity, of patients with systemic lupus erythematosus (SLE) in a single-center cohort in China. Methods A cohort of Chinese SLE patients were recruited from April 2009 to February 2010, and followed up regularly in clinic at Peking Union Medical College Hospital (PUMCH). Data for baseline, follow-up, and survival were collected, including demography, manifestations, activity, the Systemic Lupus International Collaborating/American College of Rheumatology (SLICC/ACR) Damage Index (SDI), and medications. The Kaplan-Meier method was adopted for survival analysis. Predicting and risk factors for both mortality and morbidity were evaluated by the Cox proportional hazard model. Associated factors were analyzed by the logistic regression model. Results A total of 260 patients were included at entry. The one-, three-, and five-year survival rates were 98.4%, 95.5%, and 93.8%. The proportion of patients with organ damage increased from 13.4% at baseline to 28.4% at year 6. Regression analysis showed that organ damage led to higher mortality, and organ-involved flare was associated with more future damage. Time from onset to diagnosis > 1 year, nephropathy and severe organ involvement were potential prognostic factors. Furthermore, onset age > 50 and previous organ damage were predictors for further damage. Conclusion Organ damage, severe organ involvement, and prolonged remission could be targets for the management of Chinese SLE patients to further reduce mortality. Early diagnosis, paying more attention to severe organ involvement, and preventing organ-involved flares and new organ damage would be crucially important in the future for Chinese SLE patients.

ObjectiveProtein-losing enteropathy (PLE) is a complication in some systemic lupus erythematosus (SLE) patients that is often misdiagnosed. With this study, we provide insight into clinical characteristics, laboratory characteristics, diagnostic tests, risk factors, treatment, and prognosis of the disease.MethodsA retrospective, case-control study was performed in 44 patients with SLE-related PLE (PLE group) and 88 patients with active SLE (control group) admitted to our care from January 2000−January 2012. Risk factors for SLE-related PLE were examined, and we analyzed the accuracy of single and combined laboratory characteristics in discriminating SLE-related PLE from active SLE. Serum albumin and C3 levels were measured as outcome during and after treatment with corticosteroids and immunosuppressive agents.ResultsThe PLE group had lower mean serum albumin and 24-hour urine protein levels, higher mean total plasma cholesterol levels, and greater frequencies of anti-SSA and SSB seropositivity compared with the control group. Anti-SSA seropositivity, hypoalbuminemia, and hypercholesterolemia were independent risk factors for SLE-related PLE. The simultaneous presence of serum albumin (<22 g/l) and 24-hour urine protein (<0.8 g/24 h) had high specificity, positive predictive value, negative predictive value, and positive likelihood ratio, a low negative likelihood ratio and no significant reduction in sensitivity. High dosage of glucocorticosteroid combined with cyclophosphomide were mostly prescribed for SLE-related PLE.ConclusionSLE-related PLE should be considered when an SLE patient presents with generalized edema, anti-SSA antibody seropositivity, hypercholesterolemia, severe hypoalbuminemia, and low 24-hour urine protein levels. Aggressive treatment for lupus might improve prognosis.