From the Guidelines
Minimal Change Disease (MCD) is a kidney disorder characterized by significant protein loss in urine (proteinuria) without visible changes to kidney tissue under light microscopy, and its treatment typically begins with corticosteroids such as prednisone.
Definition and Pathophysiology
MCD is the most common cause of nephrotic syndrome in children, occurring when the filtering units of the kidneys (glomeruli) become damaged, specifically affecting the podocytes that form part of the filtration barrier 1. This damage allows proteins, particularly albumin, to leak into the urine, leading to symptoms like edema (swelling), hypoalbuminemia (low blood albumin), hyperlipidemia (high blood fats), and increased risk of infections and blood clots.
Treatment
The first-line treatment for MCD is corticosteroids, with approximately 95% of children achieving urinary remission of proteinuria within 4 weeks and complete remission after an 8-week course of prednisone 1. Adults with MCD generally receive lower doses of prednisone on a per kilogram basis, and treatment should be individualized based on the therapeutic response of the patient and the need to minimize corticosteroid toxicity 1. For steroid-resistant cases, calcineurin inhibitors like cyclosporine or tacrolimus may be used 1. Other options include cyclophosphamide, mycophenolate mofetil, or rituximab.
Key Points
- MCD is a common cause of nephrotic syndrome in children
- Treatment typically begins with corticosteroids such as prednisone
- Approximately 95% of children achieve urinary remission of proteinuria within 4 weeks
- Adults with MCD may require individualized treatment to minimize corticosteroid toxicity
- Steroid-resistant cases may require calcineurin inhibitors or other immunosuppressive agents
Supportive Care
Supportive care for MCD includes sodium restriction, diuretics for edema, ACE inhibitors or ARBs to reduce proteinuria, and statins for hyperlipidemia. The disease can recur in about 30-50% of cases, but long-term kidney function is generally preserved with appropriate treatment 1.
From the Research
Definition and Characteristics of Minimal Change Disease (MCD)
- Minimal Change Disease (MCD) is a pathological condition characterized by subtle glomerular lesions causing massive and reversible proteinuria that is usually steroid sensitive 2.
- The pathologic hallmark of disease is absence of visible alterations by light microscopy and effacement of foot processes by electron microscopy 2.
- MCD is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion 2.
Treatment Options for MCD
- The mainstay of therapy is prednisone, but steroid-sensitive forms frequently relapse and this leads to a percentage of patients requiring second-line steroid-sparing immunosuppression 2.
- Other treatment options include cyclophosphamide, chlorambucil, mycophenolate, and calcineurin inhibitors 3, 4.
- Tacrolimus monotherapy without corticosteroids has been shown to be effective for the treatment of de novo minimal change disease 5.
- Rituximab (RTX) may be less likely to require a change of therapy and more likely to come off immunosuppressive drugs 6.
Clinical Trials and Research
- Clinical trials are currently in progress to investigate new mechanisms and/or molecules for the treatment of MCD, including drugs blocking the adaptive immune system and agents that affect the sieving properties of the glomerular basement membrane 4.
- Recent therapeutic advances, such as the use of anti-CD20 antibodies, have provided long-term remission off-therapy and suggest new hypotheses for disease pathogenesis 2.