Chinese SLE Treatment and Research group (CSTAR) registry 2009–2019: Major clinical characteristics of Chinese patients with systemic lupus erythematosus

Abstract Objective To describe the overall clinical characteristics of patients from the Chinese SLE Treatment and Research group (CSTAR) registry in the past 10 years. Methods CSTAR registry originated as a multicenter, consecutive, and prospective design launched in 2009. The data were collected online from 304 rheumatology centers, which covered 30 provinces in China. All data were generated and uploaded in the clinic directly without secondary collection, including demographic, clinical manifestations, disease activity (SLEDAI-2K) and organ damage evaluation (SLICC Damage Index), and lab test results. Biological samples were preserved for future study. Meanwhile, data cleaning and validation were managed by a professional backstage statistician. Results A total of 25,147 SLE patients were registered up to Dec 2019. The mean age of disease onset was 31.2 years with the age of confirmed diagnosis at 32.1 years. The male to female rate was 1:11.9. 4.6% were pediatric patients. The most common clinical presentations at entry were oral ulcer (59.4%), arthritis (55.0%), alopecia (43.22%), skin rash (40.0%), and nephritis (33.5%). The mean SLEDAI score at entry was 4 and 32.6% were in moderate to severely active disease. 66.4% and 37.8% of patients were positive for anti-ds-DNA antibody or low complement level. Additionally, 1.1% of patients were with pulmonary arterial hypertension (PAH). The prevalence rate of cerebrovascular disease was 0.3%. A total of 58.2% of patients were in clinical remission when thery were registered. Conclusions The CSTAR registry is the largest ongoing SLE registry in China so far. More than 25,000 SLE patients are registered and nearly 10,000 are in follow-up visits. This registry has provided high-quality data for future studies and will become an infrastructure for domestic and international collaborations.

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Clinical characteristics and prognosis of Chinese patients with hereditary transthyretin amyloid cardiomyopathy

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-019-1235-x ◽

2019 ◽

Vol 14 (1) ◽

Cited By ~ 2

Author(s):

Shan He ◽

Zhuang Tian ◽

Hongzhi Guan ◽

Jian Li ◽

Quan Fang ◽

...

Keyword(s):

Clinical Characteristics ◽

Disease Onset ◽

Clinical Manifestations ◽

Posterior Wall ◽

Left Ventricular ◽

Ventricular Septum ◽

Chinese Patients ◽

The Mean ◽

Amyloid Cardiomyopathy ◽

Clinical Awareness

Abstract Background Hereditary transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized progressive cardiomyopathy with heterogenous clinical manifestations that lead to its misdiagnosis and poor prognosis. This study was performed to describe the clinical characteristics and natural history of Chinese patients to improve clinical awareness of this condition. Methods In this study, we retrospectively investigated 23 patients with a confirmed diagnosis of hereditary ATTR-CM in Peking Union Medical College hospital from From January 1, 2000 to December 31, 2018. Results In all, 16 patients (69.6%) were males, the median age at disease onset was 45 (33,55) years old. The median duration from symptom onset to diagnosis was 30 (18,46) months. Phenotypes were classified as exclusively cardiac (n = 1, 4.3%) and mixed type (n = 22, 95.6%). The common mutations were Gly47Arg (7 patients [30.4%]) and Val30Ala (3 patients [13%]). Ventricular hypertrophy was observed in 23 (100%) patients, the mean thickness of the ventricular septum was 16.1 ± 3.9 mm, the mean thickness of the left ventricular posterior wall was 15.1 ± 2.8 mm. The mean left ventricle ejection fraction (LVEF) was 57.3 ± 11.9% and only 5 patients (21.7%) had LVEF < 50%. 18 (78.3%) patients had abnormal electrocardiography and the most common feature was pseudoinfarct pattern (56.5%). Overall survival at 12, 24, 36, 48, and 60 months after diagnosis was 77.8, 55.6, 38.9, 27.8, and 11.1%, respectively. Survival was better in patients with EF ≥50% than in those with EF < 50% [log Rank (Mantel-Cox), χ2 = 4.03, P = 0.045]. Conclusions The clinical characteristics of ATTR are heterogeneous: men are more likely to be affected and onset symptoms are not obvious in the heart and mainly include peripheral neuropathy and autonomic neuropathy; however, LV hypertrophy, especially a thick ventricular septum and posterior wall with preserved LVEF, are often detected on echocardiography. Abnormal ECG manifestations are common. The prognosis is poor, and patients with EF > 50% have better survival. Clinicians should be more aware of the complex clinical profile of ATTR amyloidosis to avoid misdiagnosis in practice.

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Morbidity, Mortality, and Organ Damage in Patients with Antiphospholipid Syndrome

The Journal of Rheumatology ◽

10.3899/jrheum.110800 ◽

2012 ◽

Vol 39 (3) ◽

pp. 516-523 ◽

Cited By ~ 27

Author(s):

ELEFTHERIA P. GRIKA ◽

PANAYIOTIS D. ZIAKAS ◽

ELIAS ZINTZARAS ◽

HARALAMPOS M. MOUTSOPOULOS ◽

PANAYIOTIS G. VLACHOYIANNOPOULOS

Keyword(s):

Antiphospholipid Syndrome ◽

Lupus Erythematosus ◽

Descriptive Analysis ◽

Disease Onset ◽

Clinical Manifestations ◽

Organ Damage ◽

Systemic Lupus ◽

Pregnancy Morbidity ◽

Initial Event ◽

Unit Increase

Objective.To describe morbidity, organ damage, mortality, and cause of death in patients with antiphospholipid syndrome (APS).Methods.Descriptive analysis of 135 patients. Patients were clustered according to initial event: arterial thrombosis including stroke (AT; n = 46), venous thrombosis including pulmonary emboli (VT; n = 53), or pregnancy morbidity (PM; n = 36). Disease progression according to initial event and prevalence of organ damage was observed.Results.APS occurs among young individuals (mean age 33.3 ± 11.9 yrs). One-third of the patients have APS secondary to systemic lupus erythematosus (SLE) or SLE-like disease. A broad spectrum of clinical manifestations mark the disease onset even before diagnosis. The pattern of initial presentation is preserved with regard to second event; VT is followed by VT (84%), AT is followed by AT (95%), and PM is followed by PM (88.9%). The highest morbidity is attributed to neurologic damage. PM is more likely to be followed by a second event, yet is associated with less organ damage than AT and VT. After a mean followup of 7.55 years, 29% of patients experienced organ damage and 5 died, with Systemic Lupus International Collaborating Clinics score associated with increased mortality (HR 1.31, 95% CI 1.07–1.60, p = 0.01, per 1-unit increase); hematological malignancies occurred in 2 patients after a cumulative followup of 1020 person-years. Coexistent SLE adds significant damage in patients with APS.Conclusion.APS is a disease of young individuals, who experience increased morbidity. Neurologic damage is the most common cause of morbidity. AT at presentation as well as coexistent SLE are associated with poor outcome.

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Association of the CD11b rs1143679 polymorphism with systemic lupus erythematosus in the Han Chinese population

Journal of International Medical Research ◽

10.1177/0300060517719210 ◽

2017 ◽

Vol 46 (3) ◽

pp. 1008-1014 ◽

Cited By ~ 5

Author(s):

Chunmei Li ◽

Fengqing Tong ◽

Yi Ma ◽

Kai Qian ◽

Junyu Zhang ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Chinese Population ◽

Direct Sequencing ◽

Disease Onset ◽

Clinical Manifestations ◽

Han Chinese ◽

Chinese Patients ◽

Systemic Lupus ◽

Han Chinese Population

Objective To investigate the association of the CD11b single nucleotide polymorphism (SNP) rs1143679 with systemic lupus erythematosus (SLE) in Han Chinese patients, and to clarify this association with SLE clinical manifestations. Methods PCR–restriction fragment length polymorphism and direct sequencing of CD11b rs1143679 were conducted in 584 patients with SLE and 628 healthy controls in this case–control study to compare genotype and allele frequency distributions. Correlations between CD11b genotypes and clinical manifestations were also determined. Results The frequency of the CD11b rs1143679 GA genotype was 1.89% in Han Chinese patients with SLE, which was much lower than that of European and American populations, but close to the frequency observed in individuals from Hong Kong and Thailand. The CD11b rs1143679 GA genotype was also shown to confer susceptibility to SLE (odds ratio = 4.00, 95% confidence interval = 1.11–14.41). CD11b rs1143679 was found to be significantly associated with nephritis, but not with age of disease onset, arthritis, hematological involvement, or neural lesions. Conclusion CD11b rs1143679 appears to be associated with risk for SLE in the Han Chinese population, and may play an important role in the development of lupus nephritis.

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SAT0179 ADHERENCE TO HYDROXYCHLOROQUINE INFLUENCES THE INCIDENCE OF ORGAN DAMAGE DURING FOLLOW-UP IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1656 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1031.1-1031

Author(s):

J. H. Kang ◽

S. E. Choi ◽

H. Xu ◽

D. J. Park ◽

S. S. Lee

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Damage Index ◽

Clinical Manifestations ◽

Medication Possession Ratio ◽

Organ Damage ◽

Systemic Lupus ◽

Laboratory Findings ◽

The Impact

Background:Objectives:Hydroxychloroquine (HCQ) is a cornerstone drug in patients with systemic lupus erythematosus (SLE), decreasing the risk of flares and comorbidities and improving survival. This study investigated the effects of HCQ adherence on clinical manifestations, disease activity, and organ damage in Korean patients with SLE.Methods:Data on 299 SLE patients were obtained from the Korean Lupus Network registry. Demographic variables, clinical manifestations, laboratory findings, PGA, and SLEDAI-2000 and SLICC damage index scores were recorded at the time of enrollment and repeated annually for 4 consecutive years. Patients were divided into two groups according to the level of HCQ adherence. Adherence was defined by the medication possession ratio and dichotomized as ≤ 80% vs. > 80%. Univariate and multivariate analyses were performed to assess the impact of adherence to HCQ on clinical outcomes.Results:Of the 299 patients, 31 (10.4%) showed poor drug adherence during the follow-up period. Patients with poor HCQ adherence were older (P=0.011), less insured (P=0.024), experienced lower employment (P=0.033), and had a higher rate of comorbidities such as hypertension (P=0.048) and depression (P<0.001). The non-adherent group had higher mean and changed SLICC damage index scores than the adherent group across all 4 years. In the multivariate analysis, HCQ non-adherence was significantly associated with older age (OR, 1.043; 95% CI, 1.006–1.081; P=0.021), depression (OR, 11.98; 95% CI, 1.099–130.6; P=0.042), and an annual increase in the SLICC damage index score (OR, 2.275; 95% CI, 1.369–3.779; P=0.002).Conclusion:HCQ adherence might be influenced by age and depressive mood. Additionally, the poor adherence to HCQ in SLE patients was correlated with higher cumulative organ damage. Therefore, patients with SLE should be educated to take HCQ appropriately to improve their clinical outcome in clinical practice.Disclosure of Interests:None declared

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Systemic lupus erythematosus children in Egypt: Homeland spectrum amid the global situation

Lupus ◽

10.1177/09612033211043010 ◽

2021 ◽

pp. 096120332110430

Author(s):

Nahla N Eesa ◽

Hend Abdel Nabi ◽

Rasha El Owaidy ◽

Iman Khalifa ◽

Ahmed R Radwan ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Disease Onset ◽

Damage Index ◽

Clinical Manifestations ◽

Skin Lesions ◽

Systemic Lupus ◽

Disease Characteristics ◽

Egyptian Children

Objectives This study aims to present the manifestations of juvenile systemic lupus erythematosus (JSLE) across Egypt, to focus on age at onset and gender-driven influence on disease characteristics, and to compare findings to other countries. Methods The study included 404 Egyptian children with systemic lupus erythematosus (SLE) presenting to one of the specialized rheumatology centers corresponding to 13 major governorates. Juvenile cases age was ≤ 16°years at the time of recruitment. The SLE Disease Activity Index (SLEDAI) and damage index (DI) were assessed. Results The mean age was 13.2 ± 2.4°years; 355 females and 49 males (7.2:1), and the disease duration was 2.3 ± 1.6 years, while age at disease onset was 11.1 ± 2.5°years. Their SLEDAI was 13.5 ± 12.3, and DI, 0.36 ± 0.78. The overall estimated prevalence of childhood-SLE patients in the recruited cohort in Egypt was 1/100,000 population (0.24/100000 males and 1.8/100000 females). 7.4% developed pre-pubertal SLE (≤ 7 years); 73.3%, peri-pubertal; and 19.3% during early adolescence. The differences according to age group were equal for gender and clinical manifestations except skin lesions present in 59.3% of pre-pubertal onset, 74.6% of peri-pubertal, and 84.2% of adolescents ( p = 0.029), and renal involvement in 73.8% of peripubertal, 62.1% of pre-pubertal and 58.9% of adolescents ( p = 0.03). Laboratory investigations, SLEDAI, and DI were similar among age categories. Lupus nephritis was more common in Egypt compared to JSLE from other countries. Conclusion Our large multicenter study identified that female gender influenced disease characteristics with more frequent skin involvement. Skin lesions were significantly higher in adolescents, while renal involvement in peri-pubertal children.

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Clinical and immunological pattern and outcome of Egyptian systemic lupus erythematosus patients: a single center experience

Lupus ◽

10.1177/0961203318776085 ◽

2018 ◽

Vol 27 (9) ◽

pp. 1562-1569 ◽

Cited By ~ 9

Author(s):

G A Mahmoud ◽

A A Shahin ◽

H S Zayed ◽

A Moghazy ◽

B M Eissa

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Survival Rates ◽

Disease Onset ◽

Elevated Serum ◽

Damage Index ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Oral Ulcers ◽

Increased Risk

Objective The objective of this study was to describe the clinical and immunological pattern and disease outcome in Egyptian systemic lupus erythematosus patients. Patients and methods The medical records of 770 systemic lupus erythematosus patients who were followed from 2002–2015 at Kasr Alainy Hospital, Cairo University, were retrospectively reviewed. Results There were 707 (91.8%) females. The mean age at disease onset was 22.1 ± 8.6 and the disease duration was 6.1 ± 4.5 years. The main clinical manifestations were mucocutaneous (90.8% with oral ulcers affecting 52.5%), arthritis (80.3%), nephritis (67.8%), hematologic involvement (64.9%), serositis (55.2%) and neuropsychiatric manifestations (44.3%). The frequencies of antinuclear antibodies were 94.3%, anti-dsDNA 74.8%, anti-Smith 11%, anticardiolipin antibodies 29.5% and lupus anticoagulant 19.8%. Infections, predominantly bacterial, affected 337 (43.8%) patients. Thirty-three (4.3%) patients died. The main causes of death were sepsis and disease activity. The five- and 10-year survival rates for the total cohort were 97.4% and 96.3%, respectively, and were 96% and 92%, respectively for those with nephritis ( p = 0.008). Autoimmune hemolytic anemia, thrombocytopenia, elevated serum creatinine, a higher damage index, infections, a higher glucocorticoid dose and cyclophosphamide use ≥ six months were associated with an increased risk of mortality with odds ratios of 3.69, p < 0.01; 4.12, p < 0.001; 1.54, p < 0.001; 1.43, p < 0.001; 5.08, p < 0.001; 5.04, p < 0.001 and 2.25, p = 0.03, respectively. Conclusion Compared to other cohorts, a relatively lower mean age at systemic lupus erythematosus onset and higher frequencies of oral ulcers, serositis and nephritis were found.

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Pediatric lupus in Asia

Lupus ◽

10.1177/0961203310374339 ◽

2010 ◽

Vol 19 (12) ◽

pp. 1414-1418 ◽

Cited By ~ 34

Author(s):

JL Huang ◽

KW Yeh ◽

TC Yao ◽

YL Huang ◽

HT Chung ◽

...

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Disease Onset ◽

Clinical Manifestations ◽

Organ Damage ◽

Infectious Complications ◽

Asian Patients ◽

Poor Outcomes ◽

Diffuse Proliferative Glomerulonephritis ◽

Pediatric Onset

Of all patients with systemic lupus erythematosus (SLE), 15—20% are diagnosed during childhood, with disease onset prior to the age of 16 years. Because disease expression in SLE is influenced by environment factors and differs between racial and ethnic groups. The aims of this review were to describe prevalence, clinical manifestations, common infectious complications, and outcome of pediatric-onset SLE in Asia. The prevalence of pediatric-onset SLE was 6.3—19.3 per 100,000 in Asia. The ratio of female to male was 4.7—6.2. The mean age at diagnosis of pediatric-onset SLE was 8.6—13.5 years. The most common clinical features of pediatric-onset SLE in Asia were cutaneous rashes, arthritis, hematological involvement and nephritis. The occurrence of nephritis varies from 29% to 81%. The most common histopathology of lupus nephritis was diffuse proliferative glomerulonephritis (WHO Class-IV) which occurred in 39.4—54% of case of lupus nephritis. Pediatric-onset SLE patients with infections have poor outcomes than uninfected patients. Gram-negative bacilli are the most common microorganisms responsible for bacteremia in Asian patients with SLE. Recurrent major infections predict poorer disease outcome and associated organ damage in pediatric-onset SLE. Improving the survival of SLE patients was reported in Asia in recent decades. The survival was 92% at the age of 5 years, 86% at 10 years and 79% at 15 years in children with SLE in Taiwan in 2008.

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

Lupus ◽

10.1177/0961203313499086 ◽

2013 ◽

Vol 22 (11) ◽

pp. 1192-1199 ◽

Cited By ~ 49

Author(s):

M Li ◽

W Zhang ◽

X Leng ◽

Z Li ◽

Z Ye ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Research Group ◽

Clinical Characteristics ◽

Chinese Patients ◽

Systemic Lupus

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Celiac Disease in Kosovar Albanian Children: Evaluation of Clinical Features and Diagnosis

Current Pediatric Reviews ◽

10.2174/1573396316666200116093753 ◽

2020 ◽

Vol 16 (3) ◽

pp. 241-247

Author(s):

Atifete Ramosaj-Morina ◽

Alije Keka-Sylaj ◽

Arbana Baloku Zejnullahu ◽

Lidvana Spahiu ◽

Virgjina Hasbahta ◽

...

Keyword(s):

Celiac Disease ◽

Disease Onset ◽

Clinical Manifestations ◽

Gastrointestinal Symptoms ◽

Dominant Mode ◽

Primary School Children ◽

Cross Sectional ◽

Classical Form ◽

Wide Range ◽

The Mean

Background: Celiac disease is an immune-mediated disorder characterized by variable clinical manifestations, specific antibodies, HLA-DQ2/DQ8 haplotypes, and enteropathy. Objectives: The aim of this study was to present the clinical spectrum and patterns of celiac disease in Kosovar Albanian children. Methods: A cross-sectional retrospective study was performed with Albanian children aged 0-18 years, treated for celiac disease in the Pediatric Clinic, University Clinical Center of Kosovo from 2005 to 2016. Results: During the study period, 63 children were treated for celiac disease. The mean age at diagnosis was 5.5 years (SD ± 3.31). The mean age at celiac disease onset was 3.3 years (SD ± 2.02), while the mean delay from the first symptoms indicative of celiac disease to diagnosis was 2.2 years (SD ± 2.09). More than 70% of the patients were diagnosed in the first 7 years of life, mainly presented with gastrointestinal symptoms, while primary school children and adolescents mostly showed atypical symptoms (p<0.001). The classical form of celiac disease occurred in 78% of the cases. Sixty (95%) patients carried HLA-DQ2.5, DQ2.2 and/or HLA-DQ8 heterodimers, and only three of them tested negative. Conclusions: Kosovo, as the majority of developing countries, is still facing the classical form of celiac disease as the dominant mode of presentation; as a result, most children with other forms of the celiac disease remain undiagnosed. : Physicians should be aware of the wide range of clinical presentations and utilize low testing thresholds in order to prevent potential long-term problems associated with untreated celiac disease.

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Adult systemic lupus erythematosus in Egypt: The nation-wide spectrum of 3661 patients and world-wide standpoint

Lupus ◽

10.1177/09612033211014253 ◽

2021 ◽

pp. 096120332110142

Author(s):

Tamer A Gheita ◽

Rasha Abdel Noor ◽

Esam Abualfadl ◽

Osama S Abousehly ◽

Iman I El-Gazzar ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Age Of Onset ◽

Wide Spectrum ◽

Age At Onset ◽

Disease Onset ◽

Clinical Manifestations ◽

Population Based ◽

Systemic Lupus ◽

Cross Sectional

Objective The aim of this study was to present the epidemiology, clinical manifestations and treatment pattern of systemic lupus erythematosus (SLE) in Egyptian patients over the country and compare the findings to large cohorts worldwide. Objectives were extended to focus on the age at onset and gender driven influence on the disease characteristics. Patients and method This population-based, multicenter, cross-sectional study included 3661 adult SLE patients from Egyptian rheumatology departments across the nation. Demographic, clinical, and therapeutic data were assessed for all patients. Results The study included 3661 patients; 3296 females and 365 males (9.03:1) and the median age was 30 years (17–79 years), disease duration 4 years (0–75 years) while the median age at disease onset was 25 years (4–75 years). The overall estimated prevalence of adult SLE in Egypt was 6.1/100,000 population (1.2/100,000 males and 11.3/100,000 females).There were 316 (8.6%) juvenile-onset (Jo-SLE) and 3345 adult-onset (Ao-SLE). Age at onset was highest in South and lowest in Cairo (p < 0.0001). Conclusion SLE in Egypt had a wide variety of clinical and immunological manifestations, with some similarities with that in other nations and differences within the same country. The clinical characteristics, autoantibodies and comorbidities are comparable between Ao-SLE and Jo-SLE. The frequency of various clinical and immunological manifestations varied between gender. Additional studies are needed to determine the underlying factors contributing to gender and age of onset differences.

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