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Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
Published on: June 8, 2022
Lupus nephritis in childhood
1Department of Pediatrics, College of Medicine &KKUH, King Saud University, Riyadh, Saudi Arabia.
Insights
Lupus nephritis (LN) in children is often severe, with hypertension being a key prognostic factor. While treatments have improved outcomes, disease and treatment morbidity remain significant challenges.
Area of Science:
- Pediatric Nephrology
- Immunology
- Rheumatology
Background:
- Lupus nephritis (LN) presents with varying severity, from urinalysis abnormalities to end-stage renal disease.
- Pediatric LN is frequently more severe than adult cases, with a notable risk of progression to end-stage renal disease (18-50%).
- Hypertension is a critical prognostic indicator in pediatric LN, and current complement component testing (C3, C4) may not fully reflect disease activity.
Purpose of the Study:
- To review the clinical manifestations, prognostic factors, and treatment landscape of lupus nephritis in children.
- To highlight the differences in disease presentation and severity between pediatric and adult lupus nephritis.
- To discuss the classification, histological assessment, and therapeutic options for lupus nephritis.
Main Methods:
- Review of clinical data and established literature on lupus nephritis in pediatric populations.
- Analysis of prognostic indicators, including proteinuria, nephrotic syndrome, and hypertension.
- Examination of histological classification systems and the impact of activity and chronicity indices on outcome prediction.
Main Results:
- Children with LN frequently exhibit proteinuria and nephrotic syndrome (approx. 50%), with higher rates of hypertension compared to adults.
- Histological classification and activity/chronicity indices aid in predicting outcomes.
- While renal failure is less common today, morbidity from LN and its treatments remains a concern.
Conclusions:
- Pediatric lupus nephritis requires careful management due to its severity and potential for progression.
- Treatment strategies for LN are evolving, with ongoing debate regarding optimal cytotoxic therapies.
- Renal transplantation outcomes in children with end-stage renal disease due to LN are comparable to non-lupus patients, though overall morbidity persists.
Abstract:
The manifestations of lupus nephritis (LN) range from minor abnormalities detected on urinalysis to severe renal insufficiency requiring renal replacement therapy. In children, LN is often more severe than in adults. The female to male predominance is not as marked as in adults. The risk of progression to end-stage renal disease in children is 18 to 50%. The majority of children with LN have proteinuria, while the nephrotic syndrome is seen in approximately 50% of affected patients. Children with LN have higher frequency of hypertension which is considered as the most important prognostic clinical finding. The current practice of estimation of complement components, C 3 and C 4 does not adequately reflect disease activity. There are racial differences in renal survival and response to treatment. Arab patients with LN do not exhibit a distinctive serological profile. Lupus nephritis is classified into six groups depending on the severity of the histological lesion. Transformation between the histological classes occurs frequently. Histological outcome predictions have been significantly enhanced by the addition of activity and chronicity indices. Treatment of the LN may be guided by the severity of the renal biopsy appearances. Controversy persists as to the most effective cytotoxic treatment in LN and oral or intravenous (i.v.) cyclophosphamide, azathioprine, cyclosporin, i.v. immunoglobulin, plasma exchange and recently mycophenolate mofetil have been used in different units. Today, children with LN much less commonly go into renal failure. Outcome after renal transplantation of children with end-stage renal disease caused by LN is similar to non-lupus patients. Morbidity of the disease and the treatment remain a major problem.
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