Pediatric systemic lupus erythematosus
diseaseOn this page
Also known as SLE, paediatric onsetSLE, pediatric onset
Summary
Pediatric systemic lupus erythematosus (MONDO:0019725) is a disease. A subtype of systemic lupus erythematosus — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-9 / 100 000 (United States) [Orphanet-validated]
- Phenotypes (HPO): 53
Clinical features
Epidemiology
Prevalence records
2 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.7 | United States | Validated |
| Point prevalence | 1-9 / 100 000 | 1.1 | United States | Validated |
Signs & symptoms
Clinical features (HPO)
53 HPO clinical features (Orphanet curated; top 50 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0003565 | Elevated erythrocyte sedimentation rate | Very frequent (80-99%) |
| HP:0005421 | Decreased circulating complement C3 concentration | Very frequent (80-99%) |
| HP:0045042 | Decreased circulating complement C4 concentration | Very frequent (80-99%) |
| HP:0000079 | Abnormality of the urinary system | Frequent (30-79%) |
| HP:0000083 | Renal insufficiency | Frequent (30-79%) |
| HP:0000093 | Proteinuria | Frequent (30-79%) |
| HP:0000100 | Nephrotic syndrome | Frequent (30-79%) |
| HP:0000123 | Nephritis | Frequent (30-79%) |
| HP:0000790 | Hematuria | Frequent (30-79%) |
| HP:0000951 | Abnormality of the skin | Frequent (30-79%) |
| HP:0000969 | Edema | Frequent (30-79%) |
| HP:0000988 | Skin rash | Frequent (30-79%) |
| HP:0001698 | Pericardial effusion | Frequent (30-79%) |
| HP:0001873 | Thrombocytopenia | Frequent (30-79%) |
| HP:0001882 | Leukopenia | Frequent (30-79%) |
| HP:0001888 | Lymphopenia | Frequent (30-79%) |
| HP:0001937 | Microangiopathic hemolytic anemia | Frequent (30-79%) |
| HP:0001945 | Fever | Frequent (30-79%) |
| HP:0002202 | Pleural effusion | Frequent (30-79%) |
| HP:0002716 | Lymphadenopathy | Frequent (30-79%) |
| HP:0003493 | Antinuclear antibody positivity | Frequent (30-79%) |
| HP:0003613 | Antiphospholipid antibody positivity | Frequent (30-79%) |
| HP:0011024 | Abnormality of the gastrointestinal tract | Frequent (30-79%) |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | Frequent (30-79%) |
| HP:0000155 | Oral ulcer | Occasional (5-29%) |
| HP:0000707 | Abnormality of the nervous system | Occasional (5-29%) |
| HP:0000709 | Psychosis | Occasional (5-29%) |
| HP:0001250 | Seizure | Occasional (5-29%) |
| HP:0001324 | Muscle weakness | Occasional (5-29%) |
| HP:0001369 | Arthritis | Occasional (5-29%) |
| HP:0001541 | Ascites | Occasional (5-29%) |
| HP:0002013 | Vomiting | Occasional (5-29%) |
| HP:0002027 | Abdominal pain | Occasional (5-29%) |
| HP:0002086 | Abnormality of the respiratory system | Occasional (5-29%) |
| HP:0002094 | Dyspnea | Occasional (5-29%) |
| HP:0002301 | Hemiplegia | Occasional (5-29%) |
| HP:0002315 | Headache | Occasional (5-29%) |
| HP:0002725 | Systemic lupus erythematosus | Occasional (5-29%) |
| HP:0002829 | Arthralgia | Occasional (5-29%) |
| HP:0003270 | Abdominal distention | Occasional (5-29%) |
| HP:0004372 | Reduced consciousness/confusion | Occasional (5-29%) |
| HP:0007417 | Discoid lupus rash | Occasional (5-29%) |
| HP:0025300 | Malar rash | Occasional (5-29%) |
| HP:0025343 | Lupus anticoagulant | Occasional (5-29%) |
| HP:0040319 | Dark urine | Occasional (5-29%) |
| HP:0001596 | Alopecia | Very rare (<1-4%) |
| HP:0002014 | Diarrhea | Very rare (<1-4%) |
| HP:0002463 | Language impairment | Very rare (<1-4%) |
| HP:0003453 | Antineutrophil antibody positivity | Very rare (<1-4%) |
| HP:0030880 | Raynaud phenomenon | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | pediatric systemic lupus erythematosus |
| Mondo ID | MONDO:0019725 |
| Orphanet | 93552 |
| UMLS | C1274834 |
| MedGen | 698153 |
| GARD | 0019219 |
| Is cancer (heuristic) | no |
Also known as: SLE, paediatric onset · SLE, pediatric onset
Disease family
This is a subtype of systemic lupus erythematosus. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › connective tissue disorder › rheumatic disorder › lupus erythematosus › systemic lupus erythematosus › pediatric systemic lupus erythematosus
Related subtypes (11): autosomal systemic lupus erythematosus type 16, neonatal lupus erythematosus, central nervous system lupus, bullous systemic lupus erythematosus, systemic lupus erythematosus related to C4A, systemic lupus erythematosus 17, systemic lupus erythematosus related to C1QA, systemic lupus erythematosus related to C1S, systemic lupus erythematosus 18, systemic lupus erythematosus related to C1QC, systemic lupus erythematosus related to C1QB
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 0.