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Long-term efficacy and safety of quadruple therapy in childhood diffuse proliferative lupus nephritis
Gülay Demircin1, Ayse Oner, Ozlem Erdoğan
1Department of Pediatric Nephrology, Dr Sami Ulus Children's Hospital, Ankara, Turkey. gulaydemircin@hotmail.com
Insights
This study found that a quadruple therapy protocol was effective for treating diffuse proliferative lupus nephritis (DPLN) in children, achieving high remission and renal survival rates. Hypertension and high chronicity index were poor prognostic predictors.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Diffuse proliferative lupus nephritis (DPLN) is a severe manifestation of childhood systemic lupus erythematosus (SLE).
- Understanding DPLN's clinical presentation, treatment, and prognosis is crucial for pediatric patient outcomes.
Purpose of the Study:
- To evaluate the frequency, clinical presentation, treatment, prognostic factors, and outcomes of DPLN in children.
- To assess the efficacy of a specific quadruple therapy protocol in managing pediatric DPLN.
Main Methods:
- Retrospective analysis of 46 children diagnosed with SLE between 1990 and 2004.
- 26 patients had DPLN; 19 were followed for a mean of 5.9 years.
- Treatment involved methyl prednisolone pulse, oral prednisolone, cyclophosphamide, azathioprine, and dipyridamole.
Main Results:
- 56.5% of SLE patients had DPLN, often with severe renal involvement (nephrotic syndrome, renal failure).
- Complete remission achieved in 15/19 patients; 4 developed chronic renal failure.
- Renal survival rate was 78.9% at 5, 10, and 14 years.
- Hypertension and chronicity index > 6/12 predicted poor prognosis.
Conclusions:
- The quadruple therapy protocol yielded satisfactory results for pediatric DPLN.
- This approach may avoid more aggressive, costly treatments for less severe cases.
- Early identification of prognostic factors like hypertension is important.
Abstract:
In this study, we evaluated the frequency, clinical presentation, treatment protocols, prognostic factors, and outcome in children with diffuse proliferative lupus nephritis (DPLN). Between June 1990 and December 2004, 46 patients were diagnosed to have systemic lupus erythematosus (SLE), and 26 of them (56.5%) were found to have DPLN. Renal manifestations were present in 25 patients, and the majority of them presented with severe renal findings, such as nephrotic syndrome and renal failure. All patients were given a quadruple therapy protocol including 6-12 monthly courses of methyl prednisolone pulse therapy combined with oral prednisolone, oral cyclophosphamide, azathioprine, and dipyridamole. Nineteen of these patients were regularly followed up with a mean follow-up period of 5.9 years. Complete remission was achieved in 15 of 19 patients, and chronic renal failure developed in four patients. Renal survival rate was calculated to be 78.9% at the end of 5, 10, and 14 years. Although nephrotic range proteinuria, hypoalbuminemia, renal failure, and activity index above 12/24 at presentation seemed to be associated with poor prognosis, no significant difference could be found. Hypertension and chronicity index greater than 6/12 were found to be bad prognostic predictors. We concluded that satisfactory results were achieved with our quadruple therapy protocol; thus, more aggressive and expensive therapies can be avoided and preserved for more serious and persistent diseases.
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