Childhood-onset systemic lupus erythematosus

Borgia, R. Ezequiel; Silverman, Earl D.

doi:10.1097/bor.0000000000000208

Cited by 17 publications

(9 citation statements)

References 37 publications

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“…1 Although there are commonly used biomarkers of active kidney disease and central nervous system disease, there is not a specific marker for vascular involvement. 2,3…”

Section: Introductionmentioning

confidence: 99%

Pentraxin-3 levels are associated with vasculitis and disease activity in childhood-onset systemic lupus erythematosus

Şahin

Adroviç

Barut

et al. 2017

Lupus

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Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters. We aimed to ascertain if PTX3 might be a significant mediator in cSLE and if it might indicate active vasculitis during the course of the disease. Methods Serum PTX3 levels were measured in 76 patients with cSLE and 41 healthy subjects. We have investigated its relation with disease activity, damage, clinical features, laboratory parameters and medications. Results Serum levels of PTX3 were found to be increased in cSLE compared to healthy controls (mean ± SD; 10.6 ± 8.2 ng/mL vs 2.7 ± 1.3 ng/mL, p < 0.001). PTX3 concentrations were also in correlation with SLEDAI-2K ( r = 0.57, p < 0.001). When viewed from the clinical perspective, serum PTX3 levels were significantly higher only in patients with active vasculitis ( p < 0.001), Raynaud phenomenon ( p = 0.006) and mucocutaneous manifestations ( p < 0.001). However, an association between PTX3 and age, age at disease onset, disease duration, complement levels, PedSDI score (pediatric version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), ESR, CRP, procalcitonin levels, anti-ds DNA antibody, anticardiolipin antibodies was not detected. Conclusions Patients with cSLE have increased levels of serum PTX3 compared to healthy controls. Thus, serum PTX-3 level might be a significant mediator in cSLE. Apart from these, the results support that PTX3 reflects active cutaneous vasculitis in cSLE and correlates with disease activity.

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“…1 Although there are commonly used biomarkers of active kidney disease and central nervous system disease, there is not a specific marker for vascular involvement. 2,3…”

Section: Introductionmentioning

confidence: 99%

Pentraxin-3 levels are associated with vasculitis and disease activity in childhood-onset systemic lupus erythematosus

Şahin

Adroviç

Barut

et al. 2017

Lupus

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“…Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Vital organ involvement is among the most important risk factors of morbidity and mortality in jSLE, and early diagnosis of cardiovascular and pulmonary involvement has shown to reduce the mortality rate [1][2][3].…”

Section: Introductionmentioning

confidence: 99%

Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (SLE)

Adroviç

Dedeoğlu

Şahin

et al. 2017

Bosn J of Basic Med Sci

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Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE. Nevertheless, studies on PAH among jSLE patients are still rare. The aim of this study was to assess the RV function in jSLE patients by Doppler echocardiography (Echo Doppler). We also estimated pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (mPAP) in these patients. A total of 38 jSLE patients and 40 sex- and age-matched controls were retrospectively analyzed. All patients underwent combined M-mode, cross-sectional echo, and Doppler Echo examination. The RV function was significantly impaired in jSLE patients compared to controls. PASP and mPAP were normal in 37 out of 38 patients (97.37%), however, the mean values of PASP and mPAP were significantly higher in jSLE patients compared to controls (26.90 mmHg versus 21.71 mmHg and 12.63 mmHg versus 9.89 mmHg, respectively) [p < 0.05]. Only one patient (2.6 %) had elevated mPAP (60 mmHg). The right cardiac catheterization confirmed PAH in this patient. Although PAH was detected only in one patient, there was a marked increase of PAP in our jSLE patients. Overall, PASP and mPAP were significantly higher in jSLE patients compared to healthy controls. Prospective studies with ethnically diverse cohorts could give more insight on the relevance of PAP and PHT in patients with jSLE.

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“…Patients with systemic lupus erythematosus (SLE), systemic juvenile idiopathic arthritis (sJIA), and systemic vasculitis are at high risk of frequent disease flares. Furthermore, they are immunocompromised due to the altered function of the adaptive and innate immune system in addition to the need of immunosuppressive treatment [[4], [5], [6]]. Children with rheumatic diseases may require hospitalization at the first assessment or during the disease course due to either disease flare, disease related complications, or incidental infection.…”

Section: Introductionmentioning

confidence: 99%

Outcome of children with systemic rheumatic diseases admitted to pediatric intensive care unit

Al‐Mayouf

Fallatah

AlTwajery

et al. 2019

International Journal of Pediatrics and Adolescent Medicine

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ObjectiveTo report the outcome of children with rheumatic diseases admitted to pediatric intensive care unit (PICU) in a tertiary hospital.MethodsDatabase from PICU and pediatric rheumatology section used to retrieve all candidate patients admitted to PICU from June 2000 to December 2017. Medical records of all enrolled patients reviewed for diagnosis, organ involvement, reason of PICU admission, the length of stay, and outcome.ResultsTwenty-five patients (17 female, 8 male) with total of 41 admissions identified. The median PICU stay was 13.5 days (range, 3–24). The most frequent diagnoses were systemic lupus erythematosus (SLE) (48%), followed by systemic vasculitis (16%). Most admissions were from the medical ward (68.3%), while 24.4% from the emergency room. The main reason of PICU admission was a disease flare or related complications (48.8%), followed by infection (34.2%). Fifty one percent of the admissions were due to respiratory dysfunction, 46% required non-invasive respiratory support while 38% was in need for mechanical ventilation. During PICU stay, anti-microbial agents used 90.3%, immunosuppressive treatment adjustment 75.6%, anti-hypertensive 24.4% and vasopressors 12.2%. Twelve patients (48%) fully recovered and 40% partially recovered while three patients died during their PICU stay. The causes of death were macrophage activation syndrome complicated by sepsis in two patients and advanced pulmonary hypertension in one patient.ConclusionOur data showed that children with rheumatic diseases, particularly SLE, are mostly admitted to PICU with serious disease exacerbation or severe infection. To improve the outcome, early diagnosis of critical condition and proper intervention are fundamental in reducing mortality.

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Childhood-onset systemic lupus erythematosus

Abstract: A better understanding of pathophysiology may elucidate novel molecules as therapeutic targets in cSLE. Advances in the field of biomarkers may enable a more accurate prediction of clinical outcome, optimizing early interventions and therefore improving disease prognostics.

Cited by 17 publications

References 37 publications

Pentraxin-3 levels are associated with vasculitis and disease activity in childhood-onset systemic lupus erythematosus

Pentraxin-3 levels are associated with vasculitis and disease activity in childhood-onset systemic lupus erythematosus

Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (SLE)

Outcome of children with systemic rheumatic diseases admitted to pediatric intensive care unit

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