(See also Overview of Nephrotic Syndrome.)
Minimal change disease is the most common cause of nephrotic syndrome in children 4 to 8 years (80 to 90% of childhood nephrotic syndrome), but it also occurs in adults (10 to 20% of adult nephrotic syndrome). The cause is almost always unknown, although rare cases may occur secondary to drug use (especially nonsteroidal anti-inflammatory drugs [NSAIDs]) and hematologic cancers (especially Hodgkin lymphoma).
Symptoms and Signs of Minimal Change Disease
Minimal change disease causes nephrotic syndrome, usually without hypertension or azotemia; microscopic hematuria occurs in about 20% of patients, mainly adults. Azotemia can occur in secondary cases and in patients >
Diagnosis of Minimal Change Disease
In adults with idiopathic nephrotic syndrome, renal biopsy
In children, the diagnosis can be suspected (and treatment begun) based on the following typical characteristics:
Normal renal function
Non-nephritic urine sediment
Image provided by Agnes Fogo, MD, and the American Journal of Kidney Diseases' Atlas of Renal Pathology (see www.ajkd.org).
Renal biopsy is required in adults and in children with atypical presentations. Electron microscopy demonstrates edema with diffuse swelling (effacement) of foot processes of the epithelial podocytes (see figure Electron microscopic features in immunologic glomerular disorders ). Complement and Ig deposits are absent on immunofluorescence. Although effacement is not observed in the absence of proteinuria, heavy proteinuria may occur with normal foot processes.
Electron microscopic features in immunologic glomerular disorders
Treatment of Minimal Change Disease
Corticosteroids
Corticosteroids
2
Treatment is usually continued for 1 to 2 years. However, half or more relapse, requiring treatment with the same or a different regimen.
Other immunosuppressive therapies
In corticosteroid nonresponders (< 5% of children and > 10% of adults), frequent relapsers, and corticosteroid-dependent patients, prolonged remission may be achieved with the addition of another immunosuppressive agent (1, 2
Treatment references
1. Larkins NG, Liu ID, Willis NS, et al: Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children. Cochrane Database Syst Rev 4(4):CD002290, 2020. doi: 10.1002/14651858.CD002290.pub
2. Azukaitis K, Palmer SC, Strippoli GF, et al: Interventions for minimal change disease in adults with nephrotic syndrome. Cochrane Database Syst Rev 3(3):CD001537, 2022. doi: 10.1002/14651858.CD001537.pub5
Prognosis for Minimal Change Disease
The prognosis for patients with minimal change disease who receive treatment is favorable, and > 80% of patients achieve remission. Progression to renal failure occurs in < 5 % of patients and is more common among those who do not initially respond to corticosteroids (1).
Prognosis reference
1. Tarshish P, Tobin JN, Bernstein J, et al: Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol 8(5):769-776, 1997 doi: 10.1681/ASN.V85769
Key Points
Minimal change disease accounts for most cases of nephrotic syndrome in children and is usually idiopathic.
Suspect minimal change disease in children who have sudden onset of nephrotic-range proteinuria with normal renal function and a non-nephritic urine sediment.
Confirm the diagnosis by renal biopsy in adults and atypical childhood cases.
Treat initially with corticosteroids.
The prognosis is favorable, and the majority of patients achieve remission with initial treatment.
