scholarly journals Biomarkers Associated with Organ-Specific Involvement in Juvenile Systemic Lupus Erythematosus

2021 ◽  
Vol 22 (14) ◽  
pp. 7619
Author(s):  
James Greenan-Barrett ◽  
Georgia Doolan ◽  
Devina Shah ◽  
Simrun Virdee ◽  
George A. Robinson ◽  
...  

Juvenile systemic lupus erythematosus (JSLE) is characterised by onset before 18 years of age and more severe disease phenotype, increased morbidity and mortality compared to adult-onset SLE. Management strategies in JSLE rely heavily on evidence derived from adult-onset SLE studies; therefore, identifying biomarkers associated with the disease pathogenesis and reflecting particularities of JSLE clinical phenotype holds promise for better patient management and improved outcomes. This narrative review summarises the evidence related to various traditional and novel biomarkers that have shown a promising role in identifying and predicting specific organ involvement in JSLE and appraises the evidence regarding their clinical utility, focusing in particular on renal biomarkers, while also emphasising the research into cardiovascular, haematological, neurological, skin and joint disease-related JSLE biomarkers, as well as genetic biomarkers with potential clinical applications.

Lupus ◽  
2016 ◽  
Vol 26 (3) ◽  
pp. 329-333 ◽  
Author(s):  
O O Adelowo ◽  
B H Olaosebikan ◽  
B A Animashaun ◽  
R O Akintayo

Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country.


PLoS ONE ◽  
2017 ◽  
Vol 12 (5) ◽  
pp. e0177774 ◽  
Author(s):  
Ruru Guo ◽  
Yanqing Zhou ◽  
Liangjing Lu ◽  
Lanfang Cao ◽  
Junjia Cao

Lupus ◽  
2018 ◽  
Vol 27 (13) ◽  
pp. 2069-2075 ◽  
Author(s):  
N Chottawornsak ◽  
P Rodsaward ◽  
S Suwannachote ◽  
M Rachayon ◽  
T Rattananupong ◽  
...  

Objective We aim to explore the differences of skin signs between juvenile- and adult-onset systemic lupus erythematosus and to identify their associations to the development of systemic involvement. Methods A retrospective chart review of 377 systemic lupus erythematosus patients was performed. Results In total, 171 patients with juvenile systemic lupus erythematosus and 206 with adult systemic lupus erythematosus were studied. All patients were of Southeast Asian descent. The mean duration of follow up was 8.18 ± 6.19 and 9.36 ± 7.68 years for juvenile systemic lupus erythematosus and adult systemic lupus erythematosus, respectively. At diagnosis, most patients presented with acute cutaneous lupus erythematosus, whereas chronic cutaneous lupus erythematosus was twice as common in adult systemic lupus erythematosus ( p < 0.001). The mean Systemic Lupus Erythematosus Disease Activity Index of juvenile systemic lupus erythematosus was significantly higher than that of adult systemic lupus erythematosus (14.29 ± 7.13 vs 11.27 ± 6.53). Multivariate analysis revealed the following associations in juvenile systemic lupus erythematosus: acute cutaneous lupus erythematosus and non-scarring alopecia with increased risk of arthralgia, mucosal ulcers with leukopenia, cutaneous vasculitis with seizure, and finding of granular casts. On the contrary, the associations for adult systemic lupus erythematosus were oral ulcers with arthralgia and cutaneous vasculitis with myositis. Conclusions Cutaneous signs in systemic lupus erythematosus may signal prognostic implication. Interestingly, despite similar cutaneous lesions in systemic lupus erythematosus, different ages of onset are associated with different systemic involvement.


2020 ◽  
Vol 9 (3) ◽  
pp. 712 ◽  
Author(s):  
Erkan Demirkaya ◽  
Sezgin Sahin ◽  
Micol Romano ◽  
Qing Zhou ◽  
Ivona Aksentijevich

Systemic lupus erythematosus (SLE) is a clinically and genetically heterogeneous autoimmune disease. The etiology of lupus and the contribution of genetic, environmental, infectious and hormonal factors to this phenotype have yet to be elucidated. The most straightforward approach to unravel the molecular pathogenesis of lupus may rely on studies of patients who present with early-onset severe phenotypes. Typically, they have at least one of the following clinical features: childhood onset of severe disease (<5 years), parental consanguinity, and presence of family history for autoimmune diseases in a first-degree relative. These patients account for a small proportion of patients with lupus but they inform considerable knowledge about cellular pathways contributing to this inflammatory phenotype. In recent years with the aid of new sequencing technologies, novel or rare pathogenic variants have been reported in over 30 genes predisposing to SLE and SLE-like diseases. Future studies will likely discover many more genes with private variants associated to lupus-like phenotypes. In addition, genome-wide association studies (GWAS) have identified a number of common alleles (SNPs), which increase the risk of developing lupus in adult age. Discovery of a possible shared immune pathway in SLE patients, either with rare or common variants, can provide important clues to better understand this complex disorder, it’s prognosis and can help guide new therapeutic approaches. The aim of this review is to summarize the current knowledge of the clinical presentation, genetic diagnosis and mechanisms of disease in patents with lupus and lupus-related phenotypes.


2016 ◽  
Vol 56 (3) ◽  
pp. 252-257
Author(s):  
Renan Bazuco Frittoli ◽  
Karina de Oliveira Peliçari ◽  
Bruna Siqueira Bellini ◽  
Roberto Marini ◽  
Paula Teixeira Fernandes ◽  
...  

2010 ◽  
Vol 32 (11) ◽  
pp. 3643-3646 ◽  
Author(s):  
Patrícia A. Macêdo ◽  
Carolina B. Garcia ◽  
Monique K. Schmitz ◽  
Levi H. Jales ◽  
Rosa M. R. Pereira ◽  
...  

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