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Start free with EleplanIdiopathic steroid-resistant nephrotic syndrome
ORPHA:567548Clinical syndrome
Also called Idiopathic SRNS
What it is
A rare, idiopathic nephrotic syndrome characterized by the triad of proteinuria, hypoalbuminemia and edema in patients who do not respond, or only partially respond, to the initial trial of corticosteroids. Patients may be multidrug resistant or may be sensitive to second-line immunosuppressive therapy.
Key facts
- Prevalence
- 1-9 / 1 000 000 (annual incidence)
- Age of onset
- Adolescent, Adult, Childhood
- Inheritance
- Not applicable
- Classified as
- Clinical syndrome
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%
4Common30–79%
6Sometimes5–29%
9- Abdominal pain
- Abnormal urine output
- Acute kidney injury
- Fever
- Foamy urine
- Headache
- Periorbital edema
- Respiratory tract infection
and 1 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.
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.
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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