Research Group Registry Research Articles

Chinese SLE Treatment and Research group (CSTAR) registry VII: prevalence and clinical significance of serositis in Chinese patients with systemic lupus erythematosus.

To investigate both the prevalence and clinical characteristics of serositis in Chinese patients with systemic lupus erythematosus (SLE) in a large cohort in the Chinese SLE Treatment and Research group (CSTAR) database. A prospective cross-sectional study of patients with SLE was conducted based on the data from the CSTAR registry. Serositis was defined according to the 1999 revised American College of Rheumatology (ACR) criteria for SLE - that is, pleuritis/pleural effusion and/or pericarditis/pericardial effusion detected by echocardiography, chest X-ray or chest computerized tomography (CT) scan. Peritonitis/peritoneal effusion were confirmed by abdominal ultrasonography. We analysed the prevalence and clinical associations of serositis with demographic data, organ involvements, laboratory findings and SLE disease activity. Of 2104 patients with SLE, 345 were diagnosed with serositis. The prevalence of lupus nephritis (LN), interstitial lung disease and pulmonary arterial hypertension, as well as the presence of leukocytopenia, thrombocytopenia, hypocomplementemia and anti-dsDNA antibodies was significantly higher in patients with serositis (P < 0.05). Significantly higher SLE disease activity scores were found in patients with serositis compared to those patients without serositis (P < 0.05). Lupus-related peritonitis had similar clinical manifestations and laboratory profiles as serositis caused by SLE. There is a significant association of nephropathy, interstitial lung disease, pulmonary arterial hypertension, hypocomplementemia, leukocytopenia, thrombocytopenia and elevated anti-dsDNA antibodies with serositis. The results suggest that higher SLE disease activity contributes to serositis development, and should be treated aggressively.

Chinese Systemic Lupus Erythematosus Treatment and Research Group Registry VI: Effect of Cigarette Smoking on the Clinical Phenotype of Chinese Patients with Systemic Lupus Erythematosus

ObjectivesOur 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.MethodsA 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.ResultsSmokers 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).ConclusionsCigarette 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.

Chinese SLE Treatment and Research group (CSTAR) registry: V. gender impact on Chinese patients with systemic lupus erythematosus.

Many studies have shown that differences were observed between male and female lupus patients. Although systemic lupus erythematosus (SLE) affects mostly females (female:male ratio 9:1), male SLE patients show higher mortality due to kidney and neurological disease. Currently there are limited epidemiological data concerning lupus in the Chinese population. As such, the Chinese SLE Treatment and Research group (CSTAR) developed the first online registry of Chinese lupus patients in 2009, and represents a multicenter observational study that attempts to describe and compile the major clinical characteristics of lupus in Chinese patients. To investigate the effect of gender on the phenotypes of Chinese SLE patients. Data for 2104 SLE patients were prospectively collected and included in the CSTAR registry. Patients fulfilled the 1997 American College of Rheumatology (ACR) SLE classification criteria. We conducted a cross-sectional case-control study to analyze patient clinical and laboratory data at onset and at enrollment. SLE disease activity scores (SLEDAI) were also measured at enrollment. This study included 1914 women and 190 men. Males and females showed no differences in mean ages at onset, delay of diagnosis and disease duration. Males presented more frequently with fever (p = 0.003), while musculoskeletal involvement (p = 0.001) and cytopenia (p = 0.017) was more common in females as the initial manifestation at onset of SLE. For manifestations at enrollment, males presented more frequently with fever (p = 0.005), renal disease (p = 0.019), vasculitis (p = 0.032) and neuropsychiatric lupus (p = 0.007). For cumulative manifestations at enrollment, males presented more frequently with discoid rash (p < 0.001) and neuropsychiatric lupus (p = 0.036), while less frequently with arthritis (p = 0.011). However, the laboratory data showed no significant differences between the two groups at enrollment. Males also had higher SLEDAI scores at enrollment (p = 0.002). Renal disease, vasculitis and neuropsychiatric lupus are more common in male SLE patients with higher SLEDAI scores compared to female SLE patients in China.

Concomitant Use of Immunomodulators Affects the Durability of Infliximab Therapy in Children With Crohn’s Disease

It is important to determine the effects of immunomodulators on the ability of children to remain on infliximab therapy for Crohn's disease (durability of therapy), given the potential benefits and risks of concomitant therapy-especially with thiopurines in male patients. We investigated how immunomodulatory treatment affects the durability of infliximab therapy. We collected data from the Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry, from January 2002 through August 2014, on 502 children with Crohn's disease who participated in a prospective multicenter study. Data were collected from patients who received at least a 3-dose induction regimen of infliximab, and their concomitant use of immunomodulators: no thiopurine or methotrexate treatment, treatment for 6 months or less during infliximab therapy, or treatment for more than 6 months during infliximab therapy. The probabilities (± standard error) that children remained on infliximab therapy for 1 year, 3 years, and 5 years after the treatment began were 0.84 ± 0.02, 0.69 ± 0.03, and 0.60 ± 0.03, respectively. Age, sex, and disease extent or location did not affect the durability of infliximab therapy. Greater length of concomitant use of immunomodulators was associated with increased time of infliximab therapy. The probability that patients with more than 6 months of immunomodulator use remained on infliximab therapy for 5 years was 0.70 ± 0.04, compared with 0.48 ± 0.08 for patients who did not receive immunomodulators and 0.55 ± 0.06 for patients who received immunomodulators for 6 months or less (P < .001). In boys who received immunomodulators for 6 months or more after starting infliximab, the overall durability of infliximab therapy was greater among patients receiving methotrexate than thiopurine (P < .01); the probabilities that they remained on infliximab therapy for 5 years were 0.97 ± 0.03 vs 0.58 ± 0.08, respectively. In children with Crohn's disease, concomitant treatment with an immunomodulator for more than 6 months after starting infliximab therapy increases the chances that patients will remain on infliximab. In boys, methotrexate appears to increase the durability of infliximab therapy compared with thiopurine.

Major depression diagnoses among patients with systemic sclerosis: baseline and one-month followup.

Depression is common in many rheumatic diseases and is associated with poor prognosis. No studies of patients with any rheumatic diseases, however, have assessed the stability of major depressive disorder (MDD) diagnoses over time. The objective of the present study was to assess the stability of MDD diagnoses among patients with systemic sclerosis (SSc; scleroderma), a rare autoimmune rheumatic disease, across 2 assessments approximately 1 month apart. SSc patients were recruited from 7 Canadian Scleroderma Research Group Registry sites (April 2009 to June 2012). Current (30-day) MDD was assessed with the Composite International Diagnostic Interview at baseline and approximately 1 month later. Among 309 patients with baseline assessments who received followup assessments an average of 34 days later, prevalence of 30-day MDD was 4% (95% confidence interval [95% CI] 2%-7%; n = 12) at baseline and 5% (95% CI 3%-8%; n = 16) at followup. Only 3 of 12 patients (25% [95% CI 9%-53%]) with MDD at baseline had MDD 1 month later. Most patients with SSc who meet criteria for MDD appear to experience mild, time-limited episodes of low mood that often resolve on their own without specific treatment. Consistent with international guidelines on depression management in nonpsychiatric settings, "watchful waiting" or "active monitoring" is a good strategy for SSc patients with mild depression to avoid unnecessary treatment among those whose symptoms may be transient and may resolve without medical intervention.

Incidence and associated risk factors of arterial stiffness in patients with systemic lupus erythematosus

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.

Related factors of fetal loss in Chinese women with systemic lupus erythematosus: data from Chinese SLE Treatment and Research Group registry IV.

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. 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. A 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. 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.

The Canadian systemic sclerosis oral health study II: the relationship between oral and global health-related quality of life in systemic sclerosis.

Both oral and global health-related quality of life (HRQoL) are markedly impaired in SSc. In this study we aimed to determine the degree of association between oral HRQoL and global HRQoL in SSc. Subjects were recruited from the Canadian Scleroderma Research Group registry. Global HRQoL was measured using the Medical Outcomes Trust 36-item Short Form Health Survey (SF-36) and oral HRQoL with the Oral Health Impact Profile (OHIP). The Medsger Disease Severity Score was used to determine organ involvement. Multivariate regression models determined the independent association of the OHIP with the SF-36 after adjusting for confounders. This study included 156 SSc subjects. The majority (90%) were women, with a mean age of 56 years, mean disease duration 13.8 years (s.d. 8.5) and 29% of the subjects had dcSSc. Mean total OHIP score was 40.8 (s.d. 32.4). Mean SF-36 mental component summary (MCS) score was 49.7 (s.d. 11.1) and physical component summary (PCS) score was 37.0 (s.d. 10.7). In adjusted analyses, the total OHIP score was significantly associated with the SF-36 MCS and PCS, accounting for 9.7% and 5.6% of their respective variances. Measures of disease severity were not related to OHIP score. Oral HRQoL in SSc is independently associated with global HRQoL. Oral HRQoL, however, is not related to physician-assessed disease severity. This suggests that physicians may be disregarding issues related to oral health. HRQoL is an additional dimension of HRQoL not captured by generic instruments such as the SF-36.

Liver enzyme elevations within 3 months of diagnosis of inflammatory bowel disease and likelihood of liver disease.

Inflammatory bowel disease-associated liver diseases (IBD-LDs) include autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and an overlap syndrome. Prospective unbiased multicenter data regarding the frequency of IBD-LD in patients with pediatric inflammatory bowel disease (IBD) are lacking. We examined early alanine aminotransferase (ALT) and γ-glutamyl transpeptidase (GGT) elevations in children diagnosed as having IBD and assessed the likelihood of IBD-LD. Data collected from the prospective observational Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry enrolling children of age <16 years within 30 days of diagnosis. AIH, PSC, and overlap syndrome were diagnosed using local institutional criteria. A total of 1569 subjects had liver enzymes available. Of the total, 757 had both ALT and GGT, 800 had ALT only (no GGT), and 12 had GGT only (no ALT). Overall, 29 of 1569 patients (1.8%) had IBD-LD. IBD-LD was diagnosed in 1 of 661 (0.15%) of patients with both ALT and GGT ≤ 50 IU/L compared with 21 of 42 (50%) of patients with both ALT and GGT > 50 (odds ratio 660, P < 0.0001). Of the 29 patients with IBD-LD, 21 had PSC, 2 had AIH, and 6 had overlap syndrome. IBD-LD was more common in patients with ulcerative colitis and IBD-unclassified (indeterminate colitis) than in those with Crohn disease (4% vs 0.8%, respectively, P < 0.001). Elevation of both ALT and GGT within 90 days after the diagnosis of IBD is associated with a markedly increased likelihood of IBD-LD. Both ALT and GGT levels should be measured in all of the pediatric patients newly diagnosed as having IBD.

O-12: Ten years of paediatric inflammatory bowel disease incidence in the north of Scotland

O-11 Real world outcomes of contemporary treatments in children with Crohn’s disease: observations from the pediatric IBD collaborative research group registry J. Markowitz1 *, T. Lerer2, J. Morganstern2, C. Deslandres2, G. Tomer2, M. Schaefer3, S. Kugathasan2, W. Faubion2, M. Kappelman2, B. Sudel2, M. Hitch2, D. Keljo2, A. Grossman2, R. Carvalho2, N. Leleiko2, S. Saeed2, M. Oliva-Hemker2, M. Kay2, J. Rosh2, M. Pfefferkorn2, A. Otley2, J. Rick2, D. Mack2, J. Cabrera2, A. Griffiths2, J. Hyams2. 1Cohen Children’s Medical Center of NY, Lake success, United States of America, 2The Pediatric IBD Collaborative Research Group, Hartford, United States of America, 3The Pediatric IBD Collaborative Group, Hartford, United States of America

P-031: Comparing prospective, multicenter registries on pediatric IBD from different continents; results from the Pediatric IBD Collaborative Research Group Registry and the EUROKIDS registry

P-031: Comparing prospective, multicenter registries on pediatric IBD from different continents; results from the Pediatric IBD Collaborative Research Group Registry and the EUROKIDS registry

O-11: Real world outcomes of contemporary treatments in children with Crohn’s disease: observations from the pediatric IBD collaborative research group registry

O-11 Real world outcomes of contemporary treatments in children with Crohn’s disease: observations from the pediatric IBD collaborative research group registry J. Markowitz1 *, T. Lerer2, J. Morganstern2, C. Deslandres2, G. Tomer2, M. Schaefer3, S. Kugathasan2, W. Faubion2, M. Kappelman2, B. Sudel2, M. Hitch2, D. Keljo2, A. Grossman2, R. Carvalho2, N. Leleiko2, S. Saeed2, M. Oliva-Hemker2, M. Kay2, J. Rosh2, M. Pfefferkorn2, A. Otley2, J. Rick2, D. Mack2, J. Cabrera2, A. Griffiths2, J. Hyams2. 1Cohen Children’s Medical Center of NY, Lake success, United States of America, 2The Pediatric IBD Collaborative Research Group, Hartford, United States of America, 3The Pediatric IBD Collaborative Group, Hartford, United States of America

Retrospective cohort study of methotrexate use in the treatment of pediatric Crohn's disease.

Methotrexate (MTX) use as an alternative to thiopurines in the treatment of Crohn's disease (CD) in children is increasing. This study was undertaken to assess safety and efficacy of MTX in children with CD. Patients treated with MTX with a minimum of 1-year follow-up were identified in the Pediatric IBD Collaborative Research Group Registry, a prospective inception cohort study started in 2002. The clinical efficacy and safety of MTX were analyzed retrospectively. Two hundred ninety patients treated with MTX were identified. One hundred seventy-two patients received at least 3 months of MTX without thiopurine or biologicals and had ≥1 year of follow-up. Eighty-one of 172 patients (47%) received MTX as first immunomodulator (IMM), of which 22 (27%) achieved ≥12 months of sustained clinical remission without surgery, thiopurine, biologicals, or corticosteroids. Those receiving MTX as second IMM achieved similar remission rate (35%, P = not significant). Fourteen percent received MTX as first IMM in 2002 and 60% in 2010 (P = 0.005). Disease location did not affect outcomes. MTX doses were equivalent in both groups. Fifteen percent of patients developed an alanine aminotransferase >60 international units/liter and 12% developed a white blood cell <4000 cells per microliter while on MTX. Only 4% of these discontinued MTX completely. A small group of 6 centers, which contributed only about one-third of patients with CD in the registry, contributed nearly two-thirds of the patients receiving MTX (P < 0.001). MTX use as first choice IMM is increasing in pediatric CD. MTX provided sustained clinical remission in nearly one-third of patients with minimal toxicity. There is large center-to-center variability in its use.

An assessment of the measurement equivalence of English and French versions of the Center for Epidemiologic Studies Depression (CES-D) Scale in systemic sclerosis.

ObjectivesCenter for Epidemiologic Studies Depression (CES-D) Scale scores in English- and French-speaking Canadian systemic sclerosis (SSc) patients are commonly pooled in analyses, but no studies have evaluated the metric equivalence of the English and French CES-D. The study objective was to examine the metric equivalence of the CES-D in English- and French-speaking SSc patients.MethodsThe CES-D was completed by 1007 English-speaking and 248 French-speaking patients from the Canadian Scleroderma Research Group Registry. Confirmatory factor analysis (CFA) was used to assess the factor structure in both samples. The Multiple-Indicator Multiple-Cause (MIMIC) model was utilized to assess differential item functioning (DIF).ResultsA two-factor model (Positive and Negative affect) showed excellent fit in both samples. Statistically significant, but small-magnitude, DIF was found for 3 of 20 CES-D items, including items 3 (Blues), 10 (Fearful), and 11 (Sleep). Prior to accounting for DIF, French-speaking patients had 0.08 of a standard deviation (SD) lower latent scores for the Positive factor (95% confidence interval [CI]−0.25 to 0.08) and 0.09 SD higher scores (95% CI−0.07 to 0.24) for the Negative factor than English-speaking patients. After DIF correction, there was no change on the Positive factor and a non-significant increase of 0.04 SD on the Negative factor for French-speaking patients (difference = 0.13 SD, 95% CI−0.03 to 0.28).ConclusionsThe English and French versions of the CES-D, despite minor DIF on several items, are substantively equivalent and can be used in studies that combine data from English- and French-speaking Canadian SSc patients.

Chinese SLE Treatment and Research group (CSTAR) registry: II. Prevalence and risk factors of pulmonary arterial hypertension in Chinese patients with systemic lupus erythematosus

To estimate the prevalence of pulmonary arterial hypertension (PAH) and risk factors for PAH in patients registered in the Chinese SLE Treatment and Research group (CSTAR) database, the first online registry of Chinese patients with systemic lupus erythematosus (SLE). A prospective cross-sectional study of patients with SLE was conducted using the CSTAR registry. Resting transthoracic echocardiography was used to estimate pulmonary artery pressure (PAP); PAH was defined as systolic PAP (PASP)≥40 mmHg. Patients with interstitial lung disease, valvular disease or cardiomyopathy were excluded because of disease influence on PAP. We explored potential risk factors for PAH including patient characteristics, organ involvement, laboratory findings and SLE disease activity. Of 1934 patients with SLE, 74 had PASP with 54.2±17.1 (40,106) mmHg and were diagnosed with probable PAH. The incidences of lupus nephritis, pleuritis, pericarditis, hypocomplementemia, anti-SSA, and anti-ribonucleoprotein (RNP) were significantly higher in patients with PAH than in those without (p<0.05). SLE disease activity was significantly higher in patients with PAH than in unaffected patients (p<0.05). Multivariate analysis indicated that pericarditis (odds ratio (OR)=4.248), pleuritis (OR=3.061) and anti-RNP (OR=2.559) were independent risk factors for PAH in patients with SLE (p<0.05). 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.

Chinese SLE treatment and research group registry: III. association of autoantibodies with clinical manifestations in Chinese patients with systemic lupus erythematosus.

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.

Chinese SLE Treatment and Research group (CSTAR) registry: I. Major clinical characteristics of Chinese patients with systemic lupus erythematosus

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.

Prevalence and clinical correlates of pruritus in patients with systemic sclerosis: an updated analysis of 959 patients

One previous study has estimated the prevalence of pruritus in SSc, but that study had important limitations due to a relatively small sample size. The present study updates the analyses of the previous study using a substantially larger patient sample. The objectives were to (i) document the proportion of patients who experience pruritus on most days overall and by disease duration and (ii) identify clinical correlates of pruritus. Patients from the Canadian Scleroderma Research Group Registry ≥1 year after registry enrolment were asked on two consecutive annual visits whether they had experienced pruritus in the past month on most days and underwent clinical history and medical examination. Multiple logistic regression was used to assess the association between sociodemographic and clinical variables and pruritus. Among 959 patients, 42.6% reported pruritus. Of 693 patients with data for both visits, 333 (48%) did not report pruritus at either visit, 209 (30%) reported pruritus at both visits and 151 (22%) reported pruritus at one, but not the other, visit. The presence of pruritus was independently associated with greater skin involvement [odds ratio (OR) = 1.02, 95% CI 1.00, 1.04, P = 0.017] and greater gastrointestinal involvement (OR = 1.24, 95% CI 1.04, 1.48, P = 0.018). Pruritus is common in SSc across the course of the disease and has small but statistically significant associations with the degree of skin involvement and gastrointestinal system involvement.

Systemic Sclerosis in Canada’s North American Native Population: Assessment of Clinical and Serological Manifestations

Certain North American Native (NAN) populations are known to have higher rates of systemic sclerosis (SSc) compared to non-NAN; however, little is known of the specific disease characteristics in this population in Canada. This study compares the clinical and serological manifestations of SSc in NAN and white patients. This cross-sectional, multicenter study included subjects enrolled in the Canadian Scleroderma Research Group registry between September 2004 and June 2012. Subjects were evaluated with complete medical histories, physical examinations, and self-questionnaires. Ethnicity was defined by self-report. Disease characteristics were compared between NAN and white patients and multivariate analyses were performed to determine the independent association between ethnicity and various clinical manifestations. Of 1278 patients, 1038 (81%) were white, 71 (6%) were NAN, and 169 (13%) were classified as non-white/non-NAN. There were important differences between NAN and white subjects with SSc. In multivariate analysis adjusting for socioeconomic differences and smoking status, NAN ethnicity was an independent risk factor for the severity of Raynaud phenomenon and more gastrointestinal symptoms, and was associated with a nonsignificant increase in the presence of digital ulcers. NAN patients with SSc have a distinct clinical phenotype. Our study provides a strong rationale to pursue further research into genetic and environmental determinants of SSc.

Utility of the Patient Health Questionnaire‐9 to Assess Suicide Risk in Patients With Systemic Sclerosis

Item 9 of the Patient Health Questionnaire-9 (PHQ-9), which inquires about both passive thoughts of death and active ideas of self-harm, has been used to assess suicide risk in arthritis. The objectives of this study were 1) to determine the proportion of systemic sclerosis (SSc; scleroderma) patients who responded "yes" to item 9 who endorsed active suicidal ideation in response to more direct questions during a structured clinical interview, and 2) to report whether the PHQ-8, which does not include item 9 from the PHQ-9, performs similarly to the PHQ-9. Patients were recruited from the Canadian Scleroderma Research Group Registry. The PHQ-9 and Composite International Diagnostic Interview (CIDI) depression module were administered during a phone interview. Scores on the PHQ-8 were calculated by removing item 9 from the PHQ-9. Item 9 responses were compared to suicidal ideation and intent in the last year based on the CIDI. Scores on the PHQ-8 and PHQ-9 were compared using Pearson's correlations. There were 345 patients interviewed, of whom 31 (9.0%) endorsed item 9 of the PHQ-9. Of those, based on the CIDI, 14 (45.2%) had passive thoughts of suicide or death. Only 1 patient (3.2%) had thoughts about suicide in some detail at any point in the last 12 months. The correlation between PHQ-9 and PHQ-8 scores was 0.998. Item 9 appears to identify many patients who do not report active suicidal ideation. The PHQ-8 may be a better option for assessment of depressive symptoms than the PHQ-9 in patients with SSc.