Background: Approximately 15-20% cases of systemic lupus erythematosus (SLE) are diagnosed in children. There have been a Few studies reporting the epidemiological data of pediatric-onset SLE (cSLE) in China, nei...Background: Approximately 15-20% cases of systemic lupus erythematosus (SLE) are diagnosed in children. There have been a Few studies reporting the epidemiological data of pediatric-onset SLE (cSLE) in China, neither comparing the differences between cSLE and adult-onset SLE (aSLE). The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group (CSTAR) database. Methods: We made a prospective study of 225 cSLE patients (aged 〈16 years) and 1759 patients aged 16-50 years based on CSTAR registry. We analyzed initial symptoms, clinical presentations, SLE disease activity, damages, and outcomes ofcSLE, as well as compared with aSLE patients. Results: The mean age ofcSLE patients was 12.16 ± 2.92 years, with 187 (83.1%) females. Fever (P 〈 0.001) as well as mucocutaneous (P 〈 0.001 ) and renal (P = 0.006) disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects (P 〈 0.001 ). The eSLE patients were found to present more fi'equently with malar rash (P = 0.001; odds ratio {OR], 0.624; 95% confidence interval [CI ], 0.470 0.829) but less tYequently with arthritis (P 〈 0.001 ; OR, 2.013; 95% CI, 1.512-2.679) and serositis (P = 0.030; OR, 1.629; 95% CI, 1.053 2.520). There was no significant difl'erence in SLE disease activity index scores between cSLE and aSLE groups (P = 0.478). Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients (hazard ratio 0.335 [0.170 0.658], P = 0.001). The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test (0.557, P = 0.455). Conclusions: cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difl'erence in 5- year survival between cSLE and aSLE groups.展开更多
To the Editor:Pulmonary arterial hypertension (PAH)is a hemodynamic disorder with elevated pressure of pulmonary circulation.Genetic studies in familial PAH (fPAH)and idiopathic PAH (iPAH)have discovered that transfor...To the Editor:Pulmonary arterial hypertension (PAH)is a hemodynamic disorder with elevated pressure of pulmonary circulation.Genetic studies in familial PAH (fPAH)and idiopathic PAH (iPAH)have discovered that transforming growth factor-β (TGF-β) superfamily plays an important role,and the identified mutations occur in bone morphogenetic protein type 2 receptor (BMPR2), activin receptor-like kinase type 1 (ALK1),Endoglin,and SMAD9. A genome-wide association study (GWAS)in patients without BMPR2 mutations discovered that one single-nucleotide polymorphism (SNP)rs2217560 had a significant association with i/fPAH,which located 52-kb downstream of the CBLN2 gene.展开更多
文摘Background: Approximately 15-20% cases of systemic lupus erythematosus (SLE) are diagnosed in children. There have been a Few studies reporting the epidemiological data of pediatric-onset SLE (cSLE) in China, neither comparing the differences between cSLE and adult-onset SLE (aSLE). The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group (CSTAR) database. Methods: We made a prospective study of 225 cSLE patients (aged 〈16 years) and 1759 patients aged 16-50 years based on CSTAR registry. We analyzed initial symptoms, clinical presentations, SLE disease activity, damages, and outcomes ofcSLE, as well as compared with aSLE patients. Results: The mean age ofcSLE patients was 12.16 ± 2.92 years, with 187 (83.1%) females. Fever (P 〈 0.001) as well as mucocutaneous (P 〈 0.001 ) and renal (P = 0.006) disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects (P 〈 0.001 ). The eSLE patients were found to present more fi'equently with malar rash (P = 0.001; odds ratio {OR], 0.624; 95% confidence interval [CI ], 0.470 0.829) but less tYequently with arthritis (P 〈 0.001 ; OR, 2.013; 95% CI, 1.512-2.679) and serositis (P = 0.030; OR, 1.629; 95% CI, 1.053 2.520). There was no significant difl'erence in SLE disease activity index scores between cSLE and aSLE groups (P = 0.478). Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients (hazard ratio 0.335 [0.170 0.658], P = 0.001). The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test (0.557, P = 0.455). Conclusions: cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difl'erence in 5- year survival between cSLE and aSLE groups.
基金grants from the Chinese National High Technology Research and Development Program,Ministry of Science and Technology (No.2012AA02A513)Chinese National Key Technology R&D Program (Nos.2017YFC0907601, 2017YFC0907602,and 2017YFC0907603)National Natural Science Foundation of China (Nos.81400278 and 81670054).
文摘To the Editor:Pulmonary arterial hypertension (PAH)is a hemodynamic disorder with elevated pressure of pulmonary circulation.Genetic studies in familial PAH (fPAH)and idiopathic PAH (iPAH)have discovered that transforming growth factor-β (TGF-β) superfamily plays an important role,and the identified mutations occur in bone morphogenetic protein type 2 receptor (BMPR2), activin receptor-like kinase type 1 (ALK1),Endoglin,and SMAD9. A genome-wide association study (GWAS)in patients without BMPR2 mutations discovered that one single-nucleotide polymorphism (SNP)rs2217560 had a significant association with i/fPAH,which located 52-kb downstream of the CBLN2 gene.