MCID: PDT035
MIFTS: 46

Pediatric Systemic Lupus Erythematosus

Categories: Bone diseases, Nephrological diseases, Neuronal diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Pediatric Systemic Lupus Erythematosus

MalaCards integrated aliases for Pediatric Systemic Lupus Erythematosus:

Name: Pediatric Systemic Lupus Erythematosus 58
Sle, Pediatric Onset 58

Characteristics:

Orphanet epidemiological data:

58
pediatric systemic lupus erythematosus
Prevalence: 1-9/1000000 (Australia);

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare renal diseases
Rare respiratory diseases
Rare systemic and rhumatological diseases


Summaries for Pediatric Systemic Lupus Erythematosus

MalaCards based summary : Pediatric Systemic Lupus Erythematosus, also known as sle, pediatric onset, is related to lupus erythematosus and hemolytic uremic syndrome, atypical 1. An important gene associated with Pediatric Systemic Lupus Erythematosus is IRAK1 (Interleukin 1 Receptor Associated Kinase 1), and among its related pathways/superpathways are Innate Immune System and IL-2 Pathway. The drugs Ergocalciferol and Vitamin D3 have been mentioned in the context of this disorder. Affiliated tissues include bone, skin and b cells, and related phenotypes are elevated erythrocyte sedimentation rate and decreased serum complement c3

Related Diseases for Pediatric Systemic Lupus Erythematosus

Diseases related to Pediatric Systemic Lupus Erythematosus via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 144)
# Related Disease Score Top Affiliating Genes
1 lupus erythematosus 30.4 STAT4 MX1 HMGB1 C4B C4A C3
2 hemolytic uremic syndrome, atypical 1 30.0 C4B C4A C3
3 glomerulonephritis 29.5 SPP1 C4B C4A C3
4 autoimmune disease 28.7 TNFSF13B STAT4 SPP1 KLRK1 C4A C3
5 b-cell lymphoma 28.6 TNFSF13B MYC MIR17 MDM2
6 systemic lupus erythematosus 28.0 TNFSF13B STAT4 SPP1 MX1 IRAK1 HMGB1
7 exanthem 10.4
8 juvenile dermatitis herpetiformis 10.3 C4B C4A
9 immunodeficiency due to a classical component pathway complement deficiency 10.3 C4B C4A
10 thrombocytopenia 10.3
11 purpura 10.3
12 hemolytic anemia 10.3
13 anca-associated vasculitis 10.3 HMGB1 C3
14 louse-borne relapsing fever 10.3 C4B C3
15 pericarditis 10.2
16 juvenile rheumatoid arthritis 10.2
17 vasculitis 10.2
18 tick-borne relapsing fever 10.2 C4B C4A C3
19 complement factor i deficiency 10.2 C4B C4A C3
20 complement component 3 deficiency 10.2 C4B C4A C3
21 complement component 5 deficiency 10.2 C4B C4A C3
22 antiphospholipid syndrome 10.2
23 hypersensitivity reaction type iii disease 10.2 C4B C4A C3
24 complement deficiency 10.2 C4B C4A C3
25 chorea, childhood-onset, with psychomotor retardation 10.1
26 neutropenia 10.1
27 choreatic disease 10.1
28 enthesopathy 10.1
29 acute pancreatitis 10.1
30 central nervous system disease 10.1
31 peripheral nervous system disease 10.1
32 myocarditis 10.1
33 nervous system disease 10.1
34 neuropathy 10.1
35 avascular necrosis 10.1
36 afibrinogenemia, congenital 10.1 C4B C4A C3
37 epilepsy, idiopathic generalized 3 10.1 MYC E2F1
38 blood protein disease 10.0 MYC C4B C4A
39 atherosclerosis susceptibility 10.0
40 thrombocytopenic purpura, autoimmune 10.0
41 thrombophilia due to thrombin defect 10.0
42 acrocallosal syndrome 10.0
43 anemia, autoimmune hemolytic 10.0
44 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
45 c1q deficiency 10.0
46 juvenile arthritis 10.0
47 rickets 10.0
48 hemopericardium 10.0
49 cardiac tamponade 10.0
50 pericardial effusion 10.0

Graphical network of the top 20 diseases related to Pediatric Systemic Lupus Erythematosus:



Diseases related to Pediatric Systemic Lupus Erythematosus

Symptoms & Phenotypes for Pediatric Systemic Lupus Erythematosus

Human phenotypes related to Pediatric Systemic Lupus Erythematosus:

58 31 (show top 50) (show all 55)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 elevated erythrocyte sedimentation rate 58 31 hallmark (90%) Very frequent (99-80%) HP:0003565
2 decreased serum complement c3 58 31 hallmark (90%) Very frequent (99-80%) HP:0005421
3 decreased serum complement c4 58 31 hallmark (90%) Very frequent (99-80%) HP:0045042
4 proteinuria 58 31 frequent (33%) Frequent (79-30%) HP:0000093
5 renal insufficiency 58 31 frequent (33%) Frequent (79-30%) HP:0000083
6 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
7 hematuria 58 31 frequent (33%) Frequent (79-30%) HP:0000790
8 thrombocytopenia 58 31 frequent (33%) Frequent (79-30%) HP:0001873
9 nephrotic syndrome 58 31 frequent (33%) Frequent (79-30%) HP:0000100
10 lymphopenia 58 31 frequent (33%) Frequent (79-30%) HP:0001888
11 abnormality of the gastrointestinal tract 58 31 frequent (33%) Frequent (79-30%) HP:0011024
12 lymphadenopathy 58 31 frequent (33%) Frequent (79-30%) HP:0002716
13 pleural effusion 58 31 frequent (33%) Frequent (79-30%) HP:0002202
14 pericardial effusion 58 31 frequent (33%) Frequent (79-30%) HP:0001698
15 antiphospholipid antibody positivity 58 31 frequent (33%) Frequent (79-30%) HP:0003613
16 antinuclear antibody positivity 58 31 frequent (33%) Frequent (79-30%) HP:0003493
17 nephritis 58 31 frequent (33%) Frequent (79-30%) HP:0000123
18 microangiopathic hemolytic anemia 58 31 frequent (33%) Frequent (79-30%) HP:0001937
19 increased lactate dehydrogenase level 31 frequent (33%) HP:0025435
20 arthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001369
21 reduced consciousness/confusion 58 31 occasional (7.5%) Occasional (29-5%) HP:0004372
22 muscle weakness 58 31 occasional (7.5%) Occasional (29-5%) HP:0001324
23 vomiting 58 31 occasional (7.5%) Occasional (29-5%) HP:0002013
24 dyspnea 58 31 occasional (7.5%) Occasional (29-5%) HP:0002094
25 arthralgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002829
26 ascites 58 31 occasional (7.5%) Occasional (29-5%) HP:0001541
27 abdominal pain 58 31 occasional (7.5%) Occasional (29-5%) HP:0002027
28 headache 58 31 occasional (7.5%) Occasional (29-5%) HP:0002315
29 psychosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000709
30 abdominal distention 58 31 occasional (7.5%) Occasional (29-5%) HP:0003270
31 hemiplegia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002301
32 oral ulcer 58 31 occasional (7.5%) Occasional (29-5%) HP:0000155
33 systemic lupus erythematosus 58 31 occasional (7.5%) Occasional (29-5%) HP:0002725
34 dark urine 58 31 occasional (7.5%) Occasional (29-5%) HP:0040319
35 malar rash 58 31 occasional (7.5%) Occasional (29-5%) HP:0025300
36 lupus anticoagulant 58 31 occasional (7.5%) Occasional (29-5%) HP:0025343
37 discoid lupus rash 58 31 occasional (7.5%) Occasional (29-5%) HP:0007417
38 seizure 31 occasional (7.5%) HP:0001250
39 cognitive impairment 58 31 very rare (1%) Very rare (<4-1%) HP:0100543
40 myositis 58 31 very rare (1%) Very rare (<4-1%) HP:0100614
41 chest pain 58 31 very rare (1%) Very rare (<4-1%) HP:0100749
42 alopecia 58 31 very rare (1%) Very rare (<4-1%) HP:0001596
43 diarrhea 58 31 very rare (1%) Very rare (<4-1%) HP:0002014
44 raynaud phenomenon 58 31 very rare (1%) Very rare (<4-1%) HP:0030880
45 language impairment 58 31 very rare (1%) Very rare (<4-1%) HP:0002463
46 antineutrophil antibody positivity 58 31 very rare (1%) Very rare (<4-1%) HP:0003453
47 seizures 58 Occasional (29-5%)
48 edema 58 Frequent (79-30%)
49 skin rash 58 Frequent (79-30%)
50 abnormality of the urinary system 58 Frequent (79-30%)

MGI Mouse Phenotypes related to Pediatric Systemic Lupus Erythematosus:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.91 ADIPOQ C3 C4B E2F1 IRAK1 MDM2
2 hematopoietic system MP:0005397 9.9 ADIPOQ C3 C4B E2F1 IRAK1 MDM2
3 immune system MP:0005387 9.7 ADIPOQ C3 C4B E2F1 IRAK1 MDM2
4 muscle MP:0005369 9.17 ADIPOQ C3 E2F1 IRAK1 MDM2 MYC

Drugs & Therapeutics for Pediatric Systemic Lupus Erythematosus

Drugs for Pediatric Systemic Lupus Erythematosus (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 14)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ergocalciferol Approved, Nutraceutical Phase 2 50-14-6 5280793
2
Vitamin D3 Approved, Nutraceutical Phase 2 67-97-0 5280795 6221
3
Vitamin D Approved, Nutraceutical, Vet_approved Phase 2 1406-16-2
4 Trace Elements Phase 2
5 Ergocalciferols Phase 2
6 Vitamins Phase 2
7 Hormones Phase 2
8 Interferon-alpha Phase 2
9 Nutrients Phase 2
10 Micronutrients Phase 2
11 Calcium, Dietary Phase 2
12 Calciferol Phase 2
13 Vitamin D2 Phase 2
14
Calcium Nutraceutical Phase 2 7440-70-2 271

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Vitamin D3 Effects on Immune Function in Pediatric Systemic Lupus Erythematosus (SLE) Terminated NCT01709474 Phase 2 Vitamin D3 6000 IU;Vitamin D3 400 IU
2 Bone Mineral Density of Children and Adolescents With Systemic Lupus Erythematosus Completed NCT00582465
3 Health-related Quality of Life Measure in Pediatric Lupus Completed NCT00280137

Search NIH Clinical Center for Pediatric Systemic Lupus Erythematosus

Genetic Tests for Pediatric Systemic Lupus Erythematosus

Anatomical Context for Pediatric Systemic Lupus Erythematosus

MalaCards organs/tissues related to Pediatric Systemic Lupus Erythematosus:

40
Bone, Skin, B Cells, Neutrophil, Spinal Cord, Heart, Kidney

Publications for Pediatric Systemic Lupus Erythematosus

Articles related to Pediatric Systemic Lupus Erythematosus:

(show top 50) (show all 165)
# Title Authors PMID Year
1
Genetic heterogeneity of pediatric systemic lupus erythematosus with lymphoproliferation. 61
32443356 2020
2
The status of FOXP3 gene methylation in pediatric systemic lupus erythematosus. 61
32475613 2020
3
Urticarial vasculitis in pediatric systemic lupus erythematosus. 61
32372441 2020
4
Acute pancreatitis and macrophage activation syndrome in pediatric systemic lupus erythematosus: case-based review. 61
31377830 2020
5
Hydroxychloroquine in Patients with Rheumatic Disease Complicated by COVID-19: Clarifying Target Exposures and the Need for Clinical Trials. 61
32393664 2020
6
Lupus Mesenteric Vasculitis: A Rare Initial Presentation of Pediatric Systemic Lupus Erythematosus. 61
32028306 2020
7
PTPN22 Gene Polymorphisms in Pediatric Systemic Lupus Erythematosus. 61
31232672 2020
8
Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations. 61
31771606 2019
9
The behavior of neutrophil extracellular traps and NADPH oxidative activity in pediatric systemic lupus erythematosus: relation to disease activity and lupus nephritis. 61
31030361 2019
10
Validation of the 2017 Weighted criteria compared to the 1997 ACR and the 2012 SLICC in Pediatric Systemic Lupus Erythematosus. 61
31478595 2019
11
Rituximab-associated Hypogammaglobulinemia in pediatric patients with autoimmune diseases. 61
31462263 2019
12
Diagnostic value of serum high-mobility group box-1 in pediatric systemic lupus erythematosus. 61
30938057 2019
13
Clinical manifestations, immunological features and prognosis of Chinese pediatric systemic lupus erythematosus: A single-center study. 61
30957986 2019
14
A mutation in PIK3CD gene causing pediatric systemic lupus erythematosus: A case report. 61
31045771 2019
15
Incidence and risk factors of osteomyelitis in adult and pediatric systemic lupus erythematosus: a nationwide, population-based cohort study. 61
30458691 2019
16
Unique Characteristics of Prepubertal Onset Systemic Lupus Erythematosus. 61
31263503 2019
17
The role of 5-methoxytryptophan in pediatric-onset lupus nephritis: A retrospective cohort study. 61
30630711 2018
18
Exome sequencing revealed C1Q homozygous mutation in Pediatric Systemic Lupus Erythematosus. 61
29739689 2018
19
Pediatric systemic lupus erythematosus. Retrospective analysis of clinico-laboratory parameters and their association with systemic lupus erythematosus disease activity index score. 61
30284593 2018
20
Pediatric systemic lupus erythematosus. Retrospective analysis of clinico-laboratory parameters and their association with Systemic Lupus Erythematosus Disease Activity Index score. 61
29915860 2018
21
Serum IP-10 is useful for identifying renal and overall disease activity in pediatric systemic lupus erythematosus. 61
29264699 2018
22
DNA induction of MDM2 promotes proliferation of human renal mesangial cells and alters peripheral B cells subsets in pediatric systemic lupus erythematosus. 61
29324237 2018
23
Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood. 61
29868531 2018
24
Insights Gained From the Study of Pediatric Systemic Lupus Erythematosus. 61
29922296 2018
25
Unraveling the expression of microRNA-27a* & NKG2D in peripheral blood mononuclear cells and natural killer cells of pediatric systemic lupus erythematosus patients. 61
28523761 2017
26
Efficacy and Safety of Rituximab in the Management of Pediatric Systemic Lupus Erythematosus: A Systematic Review. 61
28602379 2017
27
Carotid intima-media thickness and arterial stiffness in pediatric systemic lupus erythematosus. 61
28420050 2017
28
Reduction of CD19 autoimmunity marker on B cells of paediatric SLE patients through repressing PU.1/TNF-α/BAFF axis pathway by miR-155. 61
28683581 2017
29
Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus. 61
27837194 2017
30
Clinical characteristics of children with membranous lupus nephritis: the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. 61
27510603 2017
31
Severe disease presentation and poor outcomes among pediatric systemic lupus erythematosus patients in South Africa. 61
27488473 2017
32
An Update on Treatment and Management of Pediatric Systemic Lupus Erythematosus. 61
27747587 2016
33
Targeted B cell therapies in the treatment of adult and pediatric systemic lupus erythematosus. 61
27497253 2016
34
Acute pancreatitis as a cause of mortality in pediatric systemic lupus erythematosus: Results of a multiple cause-of-death analysis in France. 61
27079760 2016
35
Alcohol, smoking and illicit drug use in pediatric systemic lupus erythematosus patients. 61
27267641 2016
36
Comorbidities of pediatric systemic lupus erythematosus: A 6-year nationwide population-based study. 61
25066707 2016
37
Idiopathic Thrombocytopenic Purpura Masquerading Pediatric Systemic Lupus Erythematosus. 61
27057049 2016
38
Interleukin-17A rs2275913, Interleukin-17F rs763780 and rs2397084 gene polymorphisms as possible risk factors in Juvenile lupus and lupus related nephritis. 61
26515887 2016
39
Incidence and prognosis of systemic lupus erythematosus in a 35 year period in Fukushima, Japan. 61
25615939 2015
40
Pediatric systemic lupus erythematosus presenting with coronary arteritis: A case series and review of the literature. 61
25840624 2015
41
Autoantigen microarrays reveal autoantibodies associated with proliferative nephritis and active disease in pediatric systemic lupus erythematosus. 61
26081107 2015
42
Cytomegalovirus in pediatric systemic lupus erythematosus: prevalence and clinical manifestations. 61
25568145 2015
43
Practice patterns and approach to kidney biopsy in lupus: a collaboration of the Midwest Pediatric Nephrology Consortium and the Childhood Arthritis and Rheumatology Research Alliance. 61
26087651 2015
44
Targeting E2F1 and c-Myc expression by microRNA-17-5p represses interferon-stimulated gene MxA in peripheral blood mononuclear cells of pediatric systemic lupus erythematosus patients. 61
26175399 2015
45
Avascular necrosis in pediatric systemic lupus erythematosus: a brief report and review of the literature. 61
25902709 2015
46
Pediatric systemic lupus erythematosus in a single nephrology unit. 61
25758881 2015
47
Increased activation of toll-like receptors-7 and -8 of peripheral blood mononuclear cells and upregulated serum cytokines in patients with pediatric systemic lupus erythematosus. 61
26884963 2015
48
High titer anti-basement membrane antibodies in a subset of patients with pediatric systemic lupus erythematosus. 61
25926050 2015
49
Shrinking lung syndrome complicating pediatric systemic lupus erythematosus. 61
24756252 2014
50
Antiphospholipid antibodies in children with systemic lupus erythematosus: a long-term clinical and laboratory follow-up status study from northwest India. 61
23563494 2014

Variations for Pediatric Systemic Lupus Erythematosus

Expression for Pediatric Systemic Lupus Erythematosus

Search GEO for disease gene expression data for Pediatric Systemic Lupus Erythematosus.

Pathways for Pediatric Systemic Lupus Erythematosus

Pathways related to Pediatric Systemic Lupus Erythematosus according to GeneCards Suite gene sharing:

(show all 25)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.84 TNFSF13B PIK3CD MYC MX1 MDM2 KLRK1
2
Show member pathways
13.06 STAT4 SPP1 MYC IRAK1 HMGB1 E2F1
3
Show member pathways
12.79 SPP1 PIK3CD MYC MX1 MDM2 E2F1
4
Show member pathways
12.71 PIK3CD MYC MDM2 IRAK1 E2F1 C3
5 12.68 STAT4 PIK3CD MYC MDM2 E2F1
6
Show member pathways
12.55 PIK3CD MYC MX1 MDM2 IRAK1 E2F1
7
Show member pathways
12.48 STAT4 PIK3CD MYC MDM2
8
Show member pathways
12.21 MYC MIR17 MDM2 E2F1
9
Show member pathways
12.11 STAT4 SPP1 PIK3CD MYC MX1 IRAK1
10
Show member pathways
12.09 PIK3CD MYC MDM2 E2F1
11 11.95 PIK3CD MYC MDM2 E2F1
12 11.92 PIK3CD MYC MIR27A MIR17 MDM2 E2F1
13 11.88 PIK3CD MYC MDM2
14 11.87 TNFSF13B MYC E2F1
15 11.82 MYC MDM2 E2F1
16 11.77 PIK3CD MYC MDM2 HMGB1
17 11.74 C4B C4A C3
18 11.72 PIK3CD MYC E2F1
19
Show member pathways
11.65 C4B C4A C3
20 11.59 MYC MDM2 E2F1
21 11.5 MYC MDM2 HMGB1 E2F1
22 11.4 MYC MIR27A E2F1
23 11.34 IRAK1 C4B C4A C3
24 11.33 MYC MDM2 E2F1
25 10.84 MYC MDM2 E2F1

GO Terms for Pediatric Systemic Lupus Erythematosus

Cellular components related to Pediatric Systemic Lupus Erythematosus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein-containing complex GO:0032991 9.43 MYC MDM2 IRAK1 E2F1 C3 ADIPOQ
2 extracellular space GO:0005615 9.28 TNFSF13B SPP1 MIR27A MIR17 HMGB1 C4B

Biological processes related to Pediatric Systemic Lupus Erythematosus according to GeneCards Suite gene sharing:

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 signal transduction GO:0007165 10.06 TNFSF13B STAT4 SPP1 PIK3CD MX1 KLRK1
2 cytokine-mediated signaling pathway GO:0019221 9.83 STAT4 PIK3CD MYC IRAK1
3 inflammatory response GO:0006954 9.8 SPP1 PIK3CD HMGB1 C4B C4A C3
4 cellular response to lipopolysaccharide GO:0071222 9.78 MIR17 KLRK1 IRAK1 HMGB1
5 negative regulation of gene expression GO:0010629 9.77 PIK3CD MYC MIR27A MIR17 MDM2
6 regulation of complement activation GO:0030449 9.73 C4B C4A C3
7 complement activation GO:0006956 9.69 C4B C4A C3
8 cellular response to hypoxia GO:0071456 9.65 MYC MIR17 MDM2 IRAK1 E2F1
9 positive regulation of fibroblast proliferation GO:0048146 9.63 MYC MIR17 E2F1
10 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.61 MIR27A MIR17 MDM2
11 positive regulation of glial cell proliferation GO:0060252 9.57 MYC E2F1
12 innate immune response GO:0045087 9.56 PIK3CD MX1 KLRK1 IRAK1 HMGB1 C4B
13 cellular response to estrogen stimulus GO:0071391 9.55 MYC MDM2
14 negative regulation of low-density lipoprotein particle clearance GO:0010989 9.54 MIR27A MIR17
15 cellular response to testosterone stimulus GO:0071394 9.49 SPP1 MYC
16 positive regulation of apoptotic cell clearance GO:2000427 9.33 C4B C4A C3
17 immune system process GO:0002376 9.28 TNFSF13B PIK3CD MX1 KLRK1 IRAK1 HMGB1

Molecular functions related to Pediatric Systemic Lupus Erythematosus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.26 TNFSF13B SPP1 HMGB1 ADIPOQ
2 endopeptidase inhibitor activity GO:0004866 8.8 C4B C4A C3

Sources for Pediatric Systemic Lupus Erythematosus

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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