Clinical characteristics and long-term outcomes of systemic lupus erythematosus in children

Objective Treatment of Libman–Sacks (LS) endocarditis in patients with systemic lupus erythematosus (SLE) is challenging due to the lack of data. This study aimed to identify the clinical characteristics of SLE patients and LS endocarditis, and to investigate the treatment and prognosis of LS endocarditis. Methods Of all the patients with SLE who underwent echocardiography between 2010 and 2019, 11 and 29 patients with and without LS endocarditis, respectively, were included. We compared the inflammatory and thrombotic profiles between patients with and without LS endocarditis, and investigated the treatment and long-term outcome of LS endocarditis. Results No significant differences were observed in disease activity, clinical characteristics and inflammatory marker levels between patients with and without LS endocarditis. Patients with LS endocarditis had a significantly higher prevalence of antiphospholipid antibody (aPL) but a lower prevalence of SLE-specific antibody. Triple positivity of aPL was found in 72.7% and 13.8% of patients with and without LS endocarditis, respectively. Of 11 patients with LS endocarditis, six patients received anticoagulation therapy, and five patients received augmented immunosuppressive therapies. One patient who did not receive anticoagulation therapy developed cerebral infarction. Nine (82%) patients with LS endocarditis were classified as having antiphospholipid syndrome (APS). Despite the residual vegetation and valve dysfunction, surgical intervention was not required during the follow-up period of 56.8 months. Conclusion A significant correlation was observed between APS and LS endocarditis. Anticoagulation therapy should be considered to prevent thromboembolic complications in SLE patients with LS endocarditis.

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Treatment of Systemic Lupus Erythematosus

Korean Journal of Medicine ◽

10.3904/kjm.2020.95.3.162 ◽

2020 ◽

Vol 95 (3) ◽

pp. 162-169

Author(s):

Bo Young Kim ◽

Sung-Soo Kim

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Immunosuppressive Agents ◽

Organ Damage ◽

Long Term Outcomes ◽

Systemic Lupus ◽

Multiple Organs ◽

Organ Manifestations ◽

Specific Organ

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of autoantibodies that result in the inflammation of multiple organs, including the skin and musculoskeletal, renal, nervous, cardiovascular, respiratory, and gastrointestinal systems. Treatment includes the use of immunomodulatory and immunosuppressive agents to target specific organ manifestations. The treatment goal in SLE is to reduce disease activity and prevent organ damage and death. Optimal long-term outcomes require not only treatment of the disease, but also the management of comorbidities. This paper reviews treatments of SLE with the aim of improving outcomes.

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An integrated extrapolation of long-term outcomes in systemic lupus erythematosus: analysis and simulation of the Hopkins Lupus Cohort

Rheumatology ◽

10.1093/rheumatology/keu375 ◽

2014 ◽

Vol 54 (4) ◽

pp. 623-632 ◽

Cited By ~ 8

Author(s):

P. Watson ◽

A. Brennan ◽

H. Birch ◽

H. Fang ◽

M. Petri

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Long Term Outcomes ◽

Systemic Lupus

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Shrinking lung syndrome associated with systemic lupus erythematosus: A multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes

Autoimmunity Reviews ◽

10.1016/j.autrev.2016.07.021 ◽

2016 ◽

Vol 15 (10) ◽

pp. 994-1000 ◽

Cited By ~ 17

Author(s):

Loïc Duron ◽

Fleur Cohen-Aubart ◽

Elisabeth Diot ◽

Raphaël Borie ◽

Sébastien Abad ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Treatment Response ◽

Lupus Erythematosus ◽

Collaborative Study ◽

Long Term Outcomes ◽

Systemic Lupus

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Long-term outcomes in childhood-onset systemic lupus erythematosus: preliminary results of the CHILL-NL study

Pediatric Rheumatology ◽

10.1186/1546-0096-12-s1-p311 ◽

2014 ◽

Vol 12 (S1) ◽

Author(s):

Noortje Groot ◽

◽

Sylvia Kamphuis

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Long Term Outcomes ◽

Childhood Onset ◽

Systemic Lupus ◽

Preliminary Results ◽

Onset Systemic Lupus Erythematosus

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Long-term outcomes of patients with systemic lupus erythematosus: A Multicenter Cohort Study from CSTAR registry

Rheumatology and Immunology Research ◽

10.2478/rir-2021-0025 ◽

2021 ◽

Vol 2 (3) ◽

pp. 195-202

Author(s):

Ziqian Wang ◽

Mengtao Li ◽

Zhizhong Ye ◽

Caifeng Li ◽

Zhijun Li ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Logistic Regression ◽

Regression Analysis ◽

Survival Rate ◽

Lupus Erythematosus ◽

Disease Onset ◽

Organ Damage ◽

Long Term Outcomes ◽

Systemic Lupus

Abstract Objective To study the long-term outcomes, in the context of both mortality and organ damage in patients with systemic lupus erythematosus (SLE) in the Chinese SLE Treatment and Research group (CSTAR) registry cohort. Methods Patients were enrolled from April 2009 to February 2010 and they were followed up. The demographic data, clinical manifestations, labs test results and imaging examinations, disease activity (SLEDAI-2K), damage scores (SLLIC/Damage Index [SDI]), and medications were collected. Data were censored at either the last clinic visit or telephonic interview. Survival rate was analyzed by Kaplan–Meier (KM) method. COX proportional hazard model was adopted to perform the analysis of predicting factors for mortality and organ damage. Logistic regression analysis was employed to discuss the relationship among mortality, organ damage, and flare. Results A total of 2104 patients were recruited at baseline and 1494 patients were followed up. The cumulative 1-year, 3-year, and 5-year survival rates were 98.3%, 96.9%, and 95.7%, respectively. Seventy-eight patients died during follow-up, and the main causes of death were infection (34.6%), active disease (26.9%), cardiovascular and cerebrovascular events (5.13%), and malignancy (5.13%). At entry, 247 patients presented with irreversible organ damage and it increased to 398 patients at the endpoint. The major accumulated organ damages were kidney (25.9%), musculoskeletal disease (20.2%), neuropsychiatric disease (12.2%), and pulmonary damage (10.9%). Cox regression analysis further showed that male, late disease onset, delayed diagnosis (diagnosis from disease onset >1 year), baseline organ damage, and specific organ involvements predicted for higher mortality. In addition, early disease onset was a protecting factor for organ damage, and anti-SSA was an independent predicting factor for new organ damage. Logistic regression analysis showed that flare predicted for more organ damage. Conclusion The 5-year survival rate of Chinese SLE patients has improved and is comparable to Caucasians SLE patients. Disease flare impact on prognosis is the increasing risk of damage development. Early diagnosis, prevention for flare and damage to maintain remission, may improve outcome.

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