The association between initial manifestations of childhood‐onset systemic lupus erythematosus and the survival

Fatemi, Alimohammad; Matinfar, Mohammad; Saber, Mina; Smiley, Abbas

doi:10.1111/1756-185x.12807

Cited by 12 publications

(9 citation statements)

References 35 publications

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“…The prevalence of DAH in cSLE patients in the present study was similar to other reports, varying from 2 to 5% [6,7,24]. Diagnosis of this condition included the typical respiratory symptoms and signs, drop in hemoglobin levels and the radiographic evidence of pulmonary infiltrates, as well as the assessment of hypoxemia due to acute respiratory distress [7].…”

Section: Discussionsupporting

confidence: 88%

Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome

et al. 2018

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Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. Methods: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p < 0.0022). Results: DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1-151) vs. 4 (1-151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5-40) vs. 6 (0-44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients. Conclusions: This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.

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Section: Discussionsupporting

confidence: 88%

Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome

et al. 2018

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“…A few studies showed hematuria to significantly increase the mortality rate in participants with childhood-onset SLE that might have been due to complications associated with the renal organ. [ 38 ] However, other studies have concluded that patients with childhood-onset and adult-onset SLE with renal involvement should both be carefully monitored to prevent unwanted outcomes.…”

Section: Discussionmentioning

confidence: 99%

Differences in clinical features observed between childhood-onset versus adult-onset systemic lupus erythematosus

Bundhun

Kumari²,

Huang

2017

Medicine

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Background:Systemic lupus erythematosus (SLE) affects people in childhood (childhood onset) or in adulthood (adult onset). Observational studies that have previously compared childhood-onset versus adult-onset SLE were often restricted to 1 ethnic group, or to a particular area, with a small sample size of patients. We aimed to systematically compare childhood-onset versus adult-onset SLE through a meta-analysis.Methods:Electronic databases were searched for relevant publications comparing childhood-onset with adult-onset SLE. Adverse clinical features were considered as the endpoints. The Newcastle Ottawa Scale (NOS) was used to assess the methodological quality of the studies and RevMan software (version 5.3) was used to carry out this analysis whereby risk ratios (RRs) and 95% confidence intervals (95% CIs) were used as the statistical parameters.Results:A total number of 10,261 participants (1560 participants with childhood-onset SLE and 8701 participants with adult-onset SLE) were enrolled. Results of this analysis showed that compared with childhood-onset SLE, pulmonary involvement was significantly higher with adult-onset SLE (RR: 1.51, 95% CI: 1.18–1.93; P = .001), whereas renal involvement was significantly higher with childhood-onset SLE (RR: 0.65, 95% CI: 0.55–0.77; P = .00001). Raynaud phenomenon and photosensitivity were significantly higher in adult-onset SLE (RR: 1.29, 95% CI: 1.04–1.60; P = .02) and (RR: 1.08, 95% CI: 1.01–1.17; P = .03), respectively. Malar rash significantly favored adult-onset SLE (RR: 0.84, 95% CI: 0.75–0.94; P = .002). Childhood-onset SLE was associated with significantly higher hemolytic anemia, thrombocytopenia, leukocytopenia, and lymphopenia. Seizure and ocular manifestations were significantly higher with childhood-onset SLE (RR: 0.57, 95% CI: 0.47–0.70; P = .00001) and (RR: 0.34, 95% CI: 0.21–0.55; P = .00001), respectively, whereas pleuritis was significantly higher with adult-onset SLE (RR: 1.45, 95% CI: 1.17–1.79; P = .0008). Vasculitis and fever were significantly higher with childhood-onset SLE (RR: 0.51, 95% CI: 0.36–0.74; P = .0004) and (RR: 0.78, 95% CI: 0.68–0.89; P = .0002) respectively.Conclusion:Significant differences were observed between childhood-onset versus adult-onset SLE, showing the former to be more aggressive.

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“…Approximately one third of children will present with life-threatening, “non-classical” clinical manifestations that are not included in the revised American College of Rheumatology classification criteria. Fatemi et al [ 15 ] retrospectively studied 188 children, 33 were diagnosed with SLE when they were <12 years, 149 (81.9%) with SLE at 12–18 years of age, 6 patients were lost to follow-up. Both groups had similar clinical manifestations and laboratory findings.…”

Section: Reviewmentioning

confidence: 99%

Advances in paediatrics in 2016: current practices and challenges in allergy, autoimmune diseases, cardiology, endocrinology, gastroenterology, infectious diseases, neonatology, nephrology, neurology, nutrition, pulmonology

et al. 2017

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This review reports main progresses in various pediatric issues published in Italian Journal of Pediatrics and in international journals in 2016. New insights in clinical features or complications of several disorders may be useful for our better understanding. They comprise severe asthma, changing features of lupus erythematosus from birth to adolescence, celiac disease, functional gastrointestinal disorders, Moebius syndrome, recurrent pneumonia. Risk factors for congenital heart defects, Kawasaki disease have been widely investigated. New diagnostic tools are available for ascertaining brucellosis, celiac disease and viral infections. The usefulness of aCGH as first-tier test is confirmed in patients with neurodevelopmental disorders. Novel information have been provided on the safety of milk for infants. Recent advances in the treatment of common disorders, including neonatal respiratory distress syndrome, hypo-glycemia in newborns, atopic dermatitis, constipation, cyclic vomiting syndrome, nephrotic syndrome, diabetes mellitus, regurgitation, short stature, secretions in children with cerebral palsy have been reported. Antipyretics treatment has been updated by national guidelines and studies have excluded side effects (e.g. asthma risk during acetaminophen therapy). Vaccinations are a painful event and several options are reported to prevent this pain. Adverse effects due to metabolic abnormalities are reported for second generation antipsychotic drugs.

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The association between initial manifestations of childhood‐onset systemic lupus erythematosus and the survival

Abstract: cSLE survival in Iran was comparable to that in other developing countries. Baseline presentation with hematuria predominantly increased the mortality rate in cSLE. Prospective and larger studies in future may unfold other aspects of cSLE.

Cited by 12 publications

References 35 publications

Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome

Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome

Differences in clinical features observed between childhood-onset versus adult-onset systemic lupus erythematosus

Advances in paediatrics in 2016: current practices and challenges in allergy, autoimmune diseases, cardiology, endocrinology, gastroenterology, infectious diseases, neonatology, nephrology, neurology, nutrition, pulmonology

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