IgA Deficiency and Nephrotic Syndrome in Children.
Lorenza Di Genova1, Stefania Ceppi2, Maurizio Stefanelli3
1Pediatric Clinic, Department of Medical and Surgical Sciences, Università degli Studi di Perugia, 06132 Perugia, Italy. lory.digenova@gmail.com.
Summary
Selective IgA deficiency (IgAD) can complicate pediatric nephrotic syndrome. Treatment resistance in these cases may necessitate advanced therapies like anti-CD20 antibodies.
Area of Science:
- Pediatric Nephrology
- Immunology
Background:
- Selective IgA deficiency (IgAD) is the most prevalent primary immunodeficiency in Western nations.
- While IgAD-associated glomerulonephritis is documented in adults, pediatric cases with nephropathy are rare.
- This study details two pediatric cases of IgAD presenting with relapsing nephrotic syndrome.
Observation:
- Two boys, aged 4 and 2, presented with edema and weight gain.
- Laboratory findings confirmed IgAD, nephrotic proteinuria, and hypoalbuminemia.
- Initial steroid treatment for idiopathic nephrotic syndrome proved insufficient during dose tapering, leading to relapses.
Findings:
- Children with IgAD and nephrotic syndrome may exhibit complex disease courses.
- Corticosteroids alone were inadequate for managing relapses in these patients.
- Successful outcomes were achieved with human monoclonal anti-CD20 antibodies (rituximab and ofatumumab).
Implications:
- IgAD should be considered in pediatric nephrotic syndrome cases.
- Nephropathy in children with IgAD can be challenging to treat with standard therapies.
- Further research is needed to clarify the prognostic role of IgAD in pediatric nephrotic syndrome and guide personalized treatment strategies.
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