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Renal-limited vasculitis in children: a single-center retrospective long-term follow-up analysis
Insights
Pauci-immune renal limited vasculitis (RLV) in children can respond to immunosuppressive therapy, but renal scarring often occurs. Long-term follow-up is crucial for managing outcomes like renal insufficiency and hypertension.
Area of Science:
- Pediatric Nephrology
- Autoimmune Diseases
- Vasculitis Research
Background:
- Pauci-immune renal limited vasculitis (RLV) is a rare, aggressive autoimmune condition affecting the kidneys.
- Understanding the long-term renal outcomes in pediatric RLV is critical for effective management.
Purpose of the Study:
- To retrospectively analyze the renal outcomes of pediatric patients diagnosed with biopsy-proven RLV.
- To evaluate the impact of immunosuppressive therapy on renal function and long-term prognosis in children with RLV.
Main Methods:
- Retrospective analysis of 6 pediatric patients with biopsy-proven RLV.
- Review of treatment protocols including induction and maintenance immunosuppressive therapy (methylprednisolone, cyclophosphamide, prednisolone, azathioprine) and plasma exchange.
- Assessment of renal function, renal scarring on biopsy, and development of hypertension at diagnosis and during follow-up.
Main Results:
- Initial immunosuppressive therapy led to preserved or recovered renal function in 4 patients and dialysis independence in 1.
- Despite disease quiescence on repeat biopsy, all patients exhibited renal scarring.
- At follow-up, 2 patients had normal renal function, 3 had mild insufficiency, and 1 had advanced renal failure; 5 patients required hypertension treatment.
Conclusions:
- Early immunosuppressive treatment can yield favorable initial responses in pediatric RLV.
- Renal scarring is a common sequela, irrespective of initial treatment response, emphasizing the need for vigilant monitoring.
- Long-term follow-up is essential for managing chronic kidney disease progression and associated complications like hypertension in pediatric RLV survivors.
Abstract:
Pauci-immune renal limited vasculitis (RLV) is a rare and aggressive autoimmune disease. We retrospectively analyzed the renal outcome of 6 children with biopsy proven RLV. Median age at diagnosis was 10.6 years (range 7.1 - 14.5) and the median follow-up was 4.4 years (range 2.3 - 6.6). At diagnosis, 5 patients were given induction therapy (methylprednisolone + cyclophosphamide pulses) followed by maintenance treatment (prednisolone + azathioprine) while 1 patient received maintenance treatment only. After induction, 4 patients either retained or recovered normal renal function, and 1 patient, in whom short-term plasma exchange was prescribed to try to rescue her renal function, became free from dialysis. Repeated biopsy showed no disease activity; however, renal scarring was evident in all renal specimens. At last follow-up, 2 patients had normal renal function, 3 patients had mild renal insufficiency, and 1 patient had advanced renal failure. In addition, 5 patients were treated for hypertension. Our case series suggests that an initial favorable response to immunosuppressive therapy might not necessarily prevent the occurrence of renal scarring and highlights the importance of regular follow-up.
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