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Start free with EleplanPediatric systemic lupus erythematosus
ORPHA:93552Disease
Also called SLE, pediatric onset
What it is
A rare, systemic, autoimmune disease characterized by inflammation in any organ system, with onset prior to adulthood, presenting highly variable clinical manifestations, which usually have a more aggressive course and higher rate of major organ involvement than adult-onset systemic lupus erythematosus, resulting in potential damage to a variety of organs (e.g. the skin, kidneys, lungs, nervous system).
Key facts
- Prevalence
- 1-9 / 100 000 (United States)
- Age of onset
- Adolescent, Childhood, Infancy, Neonatal
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
3Common30–79%
21- Abnormality of the gastrointestinal tract
- Abnormality of the skin
- Abnormality of the urinary system
- Antinuclear antibody positivity
- Antiphospholipid antibody positivity
- Edema
- Fever
- Hematuria
- Increased circulating lactate dehydrogenase concentration
- Leukopenia
- Lymphadenopathy
- Lymphopenia
- Microangiopathic hemolytic anemia
- Nephritis
- Nephrotic syndrome
- Pericardial effusion
- Pleural effusion
- Proteinuria
- Renal insufficiency
- Skin rash
- Thrombocytopenia
Sometimes5–29%
21- Abdominal distention
- Abdominal pain
- Abnormality of the nervous system
- Abnormality of the respiratory system
- Arthralgia
- Arthritis
- Ascites
- Dark urine
and 13 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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