Search for a rare disease
Other search option(s)
Idiopathic steroid-sensitive nephrotic syndrome
Steroid-sensitive nephrotic syndrome (SSNS) is a kidney disease defined by selective proteinuria, hypoalbuminaemia and, on renal biopsy, minimal changes without immunoglobulin deposits.
ORPHA:69061Classification level: Disorder
- Synonym(s): -
- Prevalence: Unknown
- Inheritance: Unknown
- Age of onset: All ages
- ICD-10: N04.0
- OMIM: 615861
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
The annual incidence in children is 1 to 1.5 cases per 50 000 with a prevalence of 1 cases per 6 250. Conversely, the annual incidence in adults is only one case per 330 000. There is an incidence peak between the ages of 2 and 6 years.
SSNS is marked by sudden onset, oedema being the major symptom.
The aetiology is unknown.
In children, SSNS accounts for 80% of nephrotic syndrome cases, and therefore, in contrast to adult patients, kidney biopsy is not required unless the child presents with unusual clinical symptoms or fails to respond to steroid therapy.
In adults, the main differential diagnosis is membranous nephropathy.
Management and treatment
The first-line treatment involves steroid therapy (60 mg/m2 daily for 4 weeks followed by alternate-day therapy at tapering doses for up to six months). Patients who relapse may be maintained on low-dose alternate-day steroid therapy. Immunosuppressive drugs, such as cyclophosphamide, cyclosporin or mycophenolic acid, are given to steroid-dependent patients who develop symptoms of steroid toxicity including statural growth impairment, obesity, osteoporosis, cataract or psychological disturbances.
As long as the patient responds to therapy, there is no risk of progressive renal failure but patients may have a prolonged course with persistent relapses in adulthood.