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Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children
1Department of Pediatric Nephrology and Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Insights
Childhood polyarteritis nodosa (PAN) frequently affects kidneys. Combining corticosteroids with cyclophosphamide significantly improved patient outcomes and survival rates in this study.
Area of Science:
- Pediatric Rheumatology
- Nephrology
- Immunology
Background:
- Renal involvement is a frequent and serious complication of childhood polyarteritis nodosa (PAN).
- Understanding the presentation and outcomes of renal disease in pediatric PAN is crucial for effective management.
- This study analyzes the clinical course and treatment responses in children with PAN and kidney involvement.
Purpose of the Study:
- To retrospectively analyze the presentation and clinical course of 26 pediatric patients with polyarteritis nodosa (PAN) and renal involvement.
- To evaluate the effectiveness of different treatment regimens, including corticosteroids and cyclophosphamide.
- To determine the survival rates associated with renal manifestations of childhood PAN.
Main Methods:
- Retrospective analysis of 26 patients diagnosed with childhood polyarteritis nodosa (PAN) and renal involvement.
- Review of patient demographics, presenting renal symptoms (proteinuria, nephritic syndrome, nephrotic syndrome, renal failure, hypertension), and diagnostic methods (angiography).
- Assessment of treatment protocols including prednisone, cyclophosphamide, and pulse steroids, and their correlation with patient outcomes.
Main Results:
- The study included 26 patients (mean age 9.3 years; 12 boys, 14 girls) with diverse renal presentations including isolated proteinuria, nephritic syndrome, and renal failure.
- Patients treated with cyclophosphamide, in combination with corticosteroids, demonstrated significantly better outcomes compared to those not receiving it.
- The overall 1-year and 5-year survival rates were 72.5% and 60%, respectively, highlighting the severity of renal disease in PAN.
Conclusions:
- Renal disease is a serious manifestation of childhood polyarteritis nodosa (PAN) that requires immediate and aggressive therapeutic intervention.
- Combination therapy with oral cyclophosphamide and corticosteroids appears effective in improving outcomes for pediatric patients with PAN and renal involvement.
- Prompt diagnosis and treatment are essential to improve survival rates in children suffering from PAN-related kidney complications.
Abstract:
Renal involvement is common in childhood polyarteritis nodosa (PAN). We report a retrospective analysis of the presentation and clinical course of 26 patients with PAN and renal involvement. The mean age was 9.3 years (range 1-14 years) and there were 12 boys and 14 girls. Renal symptoms at presentation were as follows: 3 had isolated proteinuria, 9 had nephritic syndrome, 2 had nephritic and nephrotic components, and 10 had renal failure with one of the above features. Two patients with isolated hypertension were diagnosed by angiography and classified as classical PAN. Patients either received prednisone p.o. alone (n=9), or prednisone plus cyclophosphamide p.o. (n=11), or pulse steroids with prednisone p.o. and cyclophosphamide (n=2); 4 did not receive any treatment. Patients who were given cyclophosphamide had a significantly better outcome than those who did not. We suggest that oral cyclophosphamide therapy and corticosteroids are effective in the treatment of PAN. The overall 1-year and 5-year survival rates of the patients were 72.5% and 60%, respectively. In conclusion, renal disease is a serious manifestation of PAN necessitating prompt and aggressive treatment.