scholarly journals Difference in clinical profile between juvenile onset and adult-onset systemic lupus erythematosus: a meta-analysis

Author(s):  
Mandar Kalpesh Shah ◽  
Mihika Ashish Shah ◽  
Gayatri Anand Goghawala ◽  
Priyangi Manohar Kathayat ◽  
Parshwa Keyur Shah ◽  
...  

The aim was to systematically review the studies that compared clinical and serological variation between adult-onset systematic lupus erythematosus (aSLE) andjuvenile-onset systematic lupus erythematosus (jSLE). A comprehensive literature search was done, in various available electronic databases for relevant publication that compared juvenile onset SLE and adult onset SLE. The data of adverse clinical features, serological profile and mortality were extracted. Juvenile onset was defined as <18 years and adult onset was defined as >18 years. The methodological quality of study was assessed by Newcastle Ottawa scale (NOS) criteria and R version 3.3.1 was used for analysis and ORs and 95% CIs, were used as statistical parameter. A total of 14,920 patients; (12,230: aSLE, and 2,690: jSLE) were included. Renal involvement especially nephritis was significantly more in j-SLE OR: 2.18, 95% CI: [1.81;2.62]; I2=10.8% whereas musculoskeletal was significant in aSLE O.R: 0.64; C.I: [0.44; 0.93]; I2=83.4%. Seizure and malar rash were significantly higher in J-SLE OR:1.69, CI: [1.31; 2.18]; I2=31.1%,1.43; C.I [1.04; 1.97]; I2=82%, respectively. Raynaud’s phenomenon and pleuritis were significantly higher in adult onset SLE. Anemia and thrombocytopenia were significantly higher in juvenile onset SLE. Anti-ds DNA, anti-histone, and anti-ribosomal-P were more frequent in juvenile-onset SLE while, anti-Ro was more common in adult-onset disease. The cause of mortality was not significantly different in both groups. Renal biopsy of class III and IV combined and class V were significantly more in adult-onset SLE. SLEDAI was higher in j-SLE. Meta-analysis indicated that, regardless of many similar clinical and serological manifestations, there is still some variation between adult-onset SLE and juvenile-onset SLE. Although, SLE disease is continuum from juvenile to adult but disease aggressive in juvenile onset SLE.

2008 ◽  
Vol 68 (3) ◽  
pp. 412-415 ◽  
Author(s):  
I E A Hoffman ◽  
B R Lauwerys ◽  
F De Keyser ◽  
T W J Huizinga ◽  
D Isenberg ◽  
...  

Objective:To investigate differences in clinical signs and symptoms, and in antinuclear antibodies (ANA), between patients with juvenile-onset and adult-onset systemic lupus erythematosus (SLE).Methods:Clinical and serological data of 56 patients with juvenile-onset SLE were compared with data of 194 patients with adult-onset SLE. ANA were determined by line immunoassay and by indirect immunofluorescence on Crithidia luciliae.Results:Renal involvement, encephalopathy and haemolytic anaemia were seen, and anti-dsDNA, anti-ribosomal P and antihistone antibodies found, significantly more often in juvenile-onset SLE. Anti-dsDNA antibodies were directly associated, and anti-ribosomal P antibodies inversely associated, with renal involvement in juvenile-onset SLE. In juvenile patients with SLE and anti-dsDNA and without anti-ribosomal P antibodies the odds ratio for glomerulonephritis was 9.00; no patients with anti-ribosomal P but without anti-dsDNA had renal involvement.Conclusion:Patients with juvenile-onset SLE more often have renal involvement and encephalopathy than patients with adult-onset SLE. Anti-ribosomal P, anti-dsDNA and antihistone antibodies are more often found in patients with juvenile-onset SLE.


Lupus ◽  
2021 ◽  
pp. 096120332098425
Author(s):  
Joseph S Massias ◽  
Eve MD Smith ◽  
Eslam Al-Abadi ◽  
Kate Armon ◽  
Kathryn Bailey ◽  
...  

Systemic lupus erythematosus (SLE) is a systemic autoimmune/inflammatory disease. Patients diagnosed with juvenile-onset SLE (jSLE), when compared to individuals with adult-onset SLE, develop more severe organ involvement, increased disease activity and greater tissue and organ damage. In adult-onset SLE, clinical characteristics, pathomechanisms, disease progression and outcomes do not only vary between individuals and age groups, but also ethnicities. However, in children and young people, the influence of ethnicity on disease onset, phenotype and outcome has not been investigated in detail. In this study, we investigated clinical and laboratory characteristics in pediatric SLE patients from different ethnic backgrounds (White Caucasian, Asian, Black African/Caribbean) accessing data from a national cohort of jSLE patients (the UK JSLE Cohort Study). Among jSLE patients in the UK, ethnicity affects both the disease’s clinical course and outcomes. At diagnosis, Black African/Caribbean jSLE patients show more “classical” laboratory and clinical features when compared to White Caucasian or Asian patients. Black African/Caribbean jSLE patients exhibit more renal involvement and more frequently receive cyclophosphamide and rituximab. Studies targeting ethnicity-specific contributors to disease expression and phenotypes are necessary to improve our pathophysiological understanding, diagnosis and treatment of jSLE.


Lupus ◽  
2011 ◽  
Vol 20 (13) ◽  
pp. 1345-1355 ◽  
Author(s):  
B Livingston ◽  
A Bonner ◽  
J Pope

Objective: It is known that age at disease onset has an impact on the clinical course andoutcome of systemic lupus erythematosus (SLE); however, the precise differences in theprevalence of SLE manifestations are debated. Our objective was to conduct a systematicliterature review and meta-analysis of all studies that directly compare childhood-onset lupus with adult-onsetlupus to determine which clinical manifestations vary with age at disease onset. Methods: A comprehensive literature search of the MEDLINE/PubMed,EMBASE, CINAHL, and SCOPUS databases was conducted to identify relevant articles. Study quality was assessed using the STROBE checklist. Study sample characteristics and clinical manifestation event rates were extracted from each study. Pooled odds ratios (ORs) were calculated using the random effects method, and between-study heterogeneity was quantified using the I2 statistic. Results: Of the 484studies identified by the search strategy, 16 were included in this review. The total number of patients was 5993 adults and 905 children with SLE. Study quality was on average 16/32, ranging from 8 to 29. Several statistically significant differences were found: malar rash, ulcers/mucocutaneous involvement, renal involvement, proteinuria, urinary cellular casts, seizures, thrombocytopenia, hemolytic anemia, fever, and lymphadenopathy were more common in childhood-onset SLE with ORs ranging from 1.3 to 3.7; however, Raynaud's, pleuritis, and sicca were more common in adult-onset SLE (twice as common). Conclusions: The results of this meta-analysis suggest that some clinical manifestations of lupus are different in childhood-onset SLE and adult-onset SLE.


2017 ◽  
Vol 44 (5) ◽  
pp. 619-625 ◽  
Author(s):  
Bahar Artim-Esen ◽  
Sezgin Şahin ◽  
Erhan Çene ◽  
Yasemin Şahinkaya ◽  
Kenan Barut ◽  
...  

Objective.Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE).Methods.For the study, 719 patients (76.9%) with aSLE and 216 (23.1%) with jSLE were examined. Comparisons between the groups were made for demographic characteristics, clinical features, auto-antibody profiles, damage, and survival rates.Results.These results were significantly more frequent in jSLE: photosensitivity, malar rash, oral ulcers, renal involvement, neuropsychiatric (NP) manifestations, and autoimmune hemolytic anemia (AIHA). Of the autoantibodies, a higher frequency of anti-dsDNA and anticardiolipin IgG and IgM were observed in the jSLE group. A significant proportion of patients with aSLE had anti-Sm positivity and pleuritis. The proportion of patients with jSLE who developed organ damage was comparable to that of patients with aSLE (53% vs 47%) and the mean damage scores were similar in both groups. Renal damage was significantly more frequent in jSLE while musculoskeletal and cardiovascular system damage and diabetes mellitus were more prominent in aSLE. Comparison of survival rates of the 2 groups did not reveal any significant differences.Conclusion.We report a higher frequency in the jSLE group of renal involvement, cutaneous symptoms, oral ulcers, NP manifestations, AIHA, and anti-dsDNA positivity. A significant proportion of patients in the jSLE group had damage, most prominently in the renal domain. Our findings might support different genetic/environmental backgrounds for these 2 subgroups.


2019 ◽  
Vol 2019 ◽  
pp. 1-10 ◽  
Author(s):  
Jiwon M. Lee ◽  
Andreas Kronbichler ◽  
Se Jin Park ◽  
Seong Heon Kim ◽  
Kyoung Hee Han ◽  
...  

Introduction. Matrix metalloproteinase (MMP) is an emerging disease marker in rheumatic diseases. This is a meta-analysis aimed at systematically reviewing association between serum MMP-3 levels and systematic lupus erythematosus (SLE) activity, which sought to raise interest in MMP-3 as a putative biomarker. Methods. We conducted a meta-analysis of serum MMP-3 levels in patients with SLE and controls. We performed a PubMed search, EMBASE search, and forward search of the retrieved articles published until Oct. 1, 2018. In addition to this, we included data from a case-control study on a national pediatric SLE cohort, in which serum MMP-3 levels were measured in 11 SLE patients and 9 controls (unpublished). Subgroup analyses based on gender and disease activity were performed. Results. A total of 662 cases and 771 controls including 651 patients and 762 controls from 11 publications were studied. We observed significantly higher MMP-3 levels in SLE patients compared to healthy controls (P<0.001, Hedges’ g: 2.104, 95% CI 1.426-2.782). In subgroup analyses, we found a significant elevation of MMP-3 in the patients with nephritis compared to those without (P=0.006, Hedges’ g: 0.611, 95% CI 0.611-1.704). This finding was consistent between patients with persistent proteinuria and those without (P=0.023, Hedges’ g: 1.535, 95% CI 0.207-2.862). Meta-analysis showed no association between MMP-3 levels and gender or anti-double strand DNA antibody titer. Conclusions. Our meta-analysis demonstrated significantly higher MMP-3 levels in SLE patients than in controls and in patients with renal involvement than in those without.


Lupus ◽  
2021 ◽  
pp. 096120332110071
Author(s):  
Jiaoniu Duan ◽  
Dan Ma ◽  
Xiaoting Wen ◽  
Qianyu Guo ◽  
Jinfang Gao ◽  
...  

Objectives This meta-analysis aimed to evaluate the effectiveness of HCQ in improving the maternal and fetal outcomes in pregnancies with SLE. Methods A literature search was conducted using PubMed, MEDLINE, EMBASE, and the Cochrane database for relevant English language articles, and Wanfang, CNKI and VIP for Chinese articles, from the databases’ inception to April 30, 2020. These studies compared the maternal and/or fetal outcomes between pregnant patients with SLE who were administered HCQ during pregnancy (HCQ+ group) and those who were not administered HCQ (HCQ− group). Two investigators extracted the data and assessed the quality using the Newcastle-Ottawa Scale (NOS) and GRADE criteria independently. Odds ratio (OR) and 95% confidence intervals (CIs) were calculated. All statistical analyses were conducted using the Stata 12.0 software. Results Nine studies involving 1132 pregnancies were included in the study (3 case controls, 2 prospective cohorts, 4 retrospective cohorts). Preeclampsia, gestational hypertension, and prematurity were significantly lower in the HCQ+ group than in the HCQ− group (OR 0.35, 95% CI 0.21–0.59), (OR 0.41, 95% CI 0.19–0.89) and (OR 0.55, 95% CI 0.36–0.86), respectively. There were no significant differences in the rates of HELLP Syndrome (OR 0.88, 95% CI 0.19–3.96), gestational diabetes (OR 2.3, 95% CI 0.44–12.12), thrombotic events (OR 0.26, 95% CI 0.05–1.51), spontaneous abortion (OR 1.77, 95% CI 0.96–3.26), premature rupture of membranes (OR 0.58, 95% CI 0.24–1.39), oligohydramnios (OR 0.90, 95% CI 0.38–2.14), live birth (OR 1.22, 95% CI 0.60–2.47), stillbirth (OR 1.00, 95% CI 0.50–2.00), congenital malformation (OR 0.53, 95% CI 0.14–2.04), low birth weight (OR 0.77, 95% CI 0.43–1.39), intrauterine distress (OR 1.07, 95% CI 0.41–2.76,), intrauterine growth restriction (OR 0.57, 95% CI 0.06–5.43), or five-minute APGAR score <7 (OR 0.72, 95% CI 0.20–2.58) between the two groups. Conclusions HCQ treatment during pregnancy could reduce the risk of preeclampsia, pregnancy hypertension and prematurity in SLE patients. The certainty of evidence is high but majority of the studies included are retrospective studies and not randomized controlled trials. Therefore, the multidisciplinary management of pregnant patients with SLE should promote HCQ use, irrespective of disease activity or severity.


2015 ◽  
Vol 38 (3) ◽  
pp. 124-129
Author(s):  
Shahana A Rahman ◽  
Mohammad Imnul Islam ◽  
Manik Kumar Talukder ◽  
Mohammed Mahbubul Islam ◽  
Syed Saimul Huque ◽  
...  

Background: Systemic lupus erythematosus in children (Paediatric SLE / pSLE) may have a great variability in disease presentation. Any organ system can be involved in pSLE leading to protean clinical manifestations.To evaluate the clinical and serological presentation of pSLE in tertiary renter Bangladesh and to compare it with other populations. Methodology: It was a retrospective cohort study carried out in the department of Paediatrics, Bangabandhu Sheikh Mujib Medical University, Dhaka during the period of January 2005 to December 2013. A total of 70 patients fulfilling the 1997 revised ACR classification criteria for SLE were enrolled in the study. Data was collected in a pre-designed questionnaire. Results: Age range was 2.5 years to 16 years, female: male ratio was 7:1. Duration of disease was 1 month to 18 months. Common clinical presentation included general weakness/fatigue (91%), fever (83%), arthralgia/arthritis (74%), oral ulcer (73%) and skin rash (71%). All the patients were anemic. ANA was positive in 97% cases and anti-ds DNA in 91% cases. More than 65% cases had evidences of renal involvement at the presentation. Among the patients who underwent kidney biopsy, 40% had diffuse proliferative lupus nephritis (WHO class IV) followed by class III in 26% cases. Conclusion: Pediatric SLE patients in our country, compared to other countries had much higher incidence of fever and non-specific complaints such as, general weakness and malaise as presenting symptoms. Thus, a strong index of suspicion should be maintained for early diagnosis of pSLE especially among adolescent girls. DOI: http://dx.doi.org/10.3329/bjch.v38i3.22819 Bangladesh J Child Health 2014; VOL 38 (3) :124-129


Lupus ◽  
2017 ◽  
Vol 27 (1) ◽  
pp. 6-16 ◽  
Author(s):  
M Pejchinovski ◽  
J Siwy ◽  
W Mullen ◽  
H Mischak ◽  
M A Petri ◽  
...  

Background Systematic lupus erythematosus (SLE) is characterized with various complications which can cause serious organ damage in the human body. Despite the significant improvements in disease management of SLE patients, the non-invasive diagnosis is entirely missing. In this study, we used urinary peptidomic biomarkers for early diagnosis of disease onset to improve patient risk stratification, vital for effective drug treatment. Methods Urine samples from patients with SLE, lupus nephritis (LN) and healthy controls (HCs) were analyzed using capillary electrophoresis coupled to mass spectrometry (CE-MS) for state-of-the-art biomarker discovery. Results A biomarker panel made up of 65 urinary peptides was developed that accurately discriminated SLE without renal involvement from HC patients. The performance of the SLE-specific panel was validated in a multicentric independent cohort consisting of patients without SLE but with different renal disease and LN. This resulted in an area under the receiver operating characteristic (ROC) curve (AUC) of 0.80 ( p < 0.0001, 95% confidence interval (CI) 0.65–0.90) corresponding to a sensitivity and a specificity of 83% and 73%, respectively. Based on the end terminal amino acid sequences of the biomarker peptides, an in silico methodology was used to identify the proteases that were up or down-regulated. This identified matrix metalloproteinases (MMPs) as being mainly responsible for the peptides fragmentation. Conclusions A laboratory-based urine test was successfully established for early diagnosis of SLE patients. Our approach determined the activity of several proteases and provided novel molecular information that could potentially influence treatment efficacy.


Lupus ◽  
2020 ◽  
Vol 29 (6) ◽  
pp. 607-616
Author(s):  
Asmaa SM Abdel-Rehim ◽  
Nesrine A Mohamed ◽  
Marwa M Shakweer

Background Lupus nephritis (LN) is an ominous manifestation of systemic lupus erythematosus (SLE). Clinical renal affection is present in about 70% of lupus patients, and more patients have histological evidence of renal involvement without clinical manifestations. This study aimed to investigate the potential role of serum interleukin-34 (IL-34) as an early marker for the detection of silent LN. Methods Thirty-three lupus patients with silent LN (group I), 37 patients with clinical LN (group II) and 20 controls were included. The SLE Disease Activity Index (SLEDAI), IL-34, anti-dsDNA antibodies and renal biopsy were assessed in all patients. Results Serum IL-34 levels were significantly higher in all lupus patients compared to healthy controls ( p < 0.001) and showed a significant positive correlation with SLEDAI score. SLE patients with positive anti-dsDNA antibodies had more active disease according to SLEDAI and higher levels of IL-34 than those with negative anti-dsDNA antibodies. In both studied groups, serum IL-34 levels were significantly higher in patients with proliferative LN (class III and class IV) than those with non-proliferative lupus (class II and class V) and controls. Yet, in both groups, IL-34 was not useful in differentiating active from chronic renal affection. Conclusion In lupus patients with insignificant proteinuria, serum levels of IL-34 distinguished the different histological classes of subclinical LN. Serum IL-34 may be used as a surrogate marker for early renal affection in silent LN, especially the proliferative type.


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