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Treatment of lupus nephritis in children
1Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, 149, rue de Sèvres, F-75015 Paris, France. niaudet@necker.fr
Insights
Pediatric lupus nephritis requires tailored treatment based on disease severity. Aggressive therapies like corticosteroids and cyclophosphamide are used for severe cases, while milder forms need careful monitoring to avoid side effects.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Systemic lupus erythematosus (SLE) in children is often more severe than in adults, with significant renal involvement (lupus nephritis) in 30%-70% of cases.
- Untreated lupus nephritis can lead to rapid renal failure and death, but aggressive treatments carry risks like growth retardation and infections.
Purpose of the Study:
- To outline treatment strategies for pediatric lupus nephritis based on histological classification and clinical presentation.
- To balance treatment efficacy with minimizing adverse effects in children with SLE.
Main Methods:
- Classification of lupus nephritis into six classes based on renal biopsy findings.
- Treatment protocols vary from careful monitoring for mild lesions (Class I/II) to aggressive regimens for severe disease (Class III/IV/V with nephrotic syndrome).
- Current protocols involve methylprednisolone pulses, oral prednisone, cyclophosphamide, and potential maintenance with azathioprine.
Main Results:
- Treatment is guided by disease severity, with mild cases (Class I/II) managed conservatively.
- Aggressive therapy, including corticosteroids and cyclophosphamide, is used for focal (Class III) and diffuse proliferative (Class IV) glomerulonephritis.
- Class V membranous glomerulonephritis requires aggressive treatment if nephrotic syndrome is present.
Conclusions:
- Tailored treatment based on lupus nephritis class and clinical symptoms is crucial for optimal outcomes in children.
- Renal transplantation is a viable option for end-stage renal disease, with rare recurrence rates.
Abstract:
In children, systemic lupus erythematosus (SLE) is often more severe than in adults. Renal disease is very common in SLE, with clinical symptoms of renal involvement occurring in 30%-70% of patients. In the absence of appropriate treatment the child may die from the disease or progress rapidly to renal failure. However, aggressive treatment regimens, in particular corticosteroids, carry the risk of growth retardation, accelerated atherosclerosis, and severe infectious complications. Lupus nephritis is classified into six groups depending on the severity of the histological lesions. The most-appropriate treatment for optimal efficacy with minimal side-effects depends on the disease severity. Mild lesions (class I or II) require only careful follow-up to identify any disease progression. Patients with class III nephropathy (focal and segmental glomerulonephritis) may have mild clinical symptoms, in which case no specific therapy is indicated, or more-severe symptoms of the nephrotic syndrome, hypertension, and sometimes moderate renal insufficiency. These patients require the same aggressive therapy as those with class IV disease (diffuse proliferative glomerulonephritis). Our current protocol starts with three methylprednisolone pulses followed by 1.5 mg/kg per day oral prednisone and six monthly pulses of cyclophosphamide. After a second renal biopsy the patient may be maintained on azathioprine while the prednisone dosage is slowly tapered. In children with milder disease we use lower doses of oral prednisone (1-1.5 mg/kg per day). Patients with membranous glomerulonephritis (class V) require no specific therapy if they have pure membranous nephropathy, but require aggressive therapy if they have the nephrotic syndrome. In those patients who progress to end-stage renal disease, clinical and serological remission is common and renal transplantation can be performed, as recurrence in the transplant is very rare.