IgA Deficiency and Nephrotic Syndrome in Children

Lorenza Di Genova, Stefania Ceppi, Maurizio Stefanelli and Susanna Esposito
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Lorenza Di Genova: Pediatric Clinic, Department of Medical and Surgical Sciences, Università degli Studi di Perugia, 06132 Perugia, Italy
Stefania Ceppi: Pediatric Clinic, Department of Medical and Surgical Sciences, Università degli Studi di Perugia, 06132 Perugia, Italy
Maurizio Stefanelli: Pediatric Clinic, Department of Medical and Surgical Sciences, Università degli Studi di Perugia, 06132 Perugia, Italy
Susanna Esposito: Pediatric Clinic, Department of Medical and Surgical Sciences, Università degli Studi di Perugia, 06132 Perugia, Italy

IJERPH, 2018, vol. 15, issue 8, 1-5

Abstract: Background : Imunoglobulin A (IgA) deficiency (IgAD) is the most common form of primary immunodeficiency in Western countries. There have been several reports on IgAD complicated by glomerulonephritis in adults, but only very few cases of IgAD with nephropathy have been reported in children. We present two cases of IgAD with relapsing nephrotic syndrome in pediatric age. Case presentation : A 4-year-old boy and a 2-year-old boy presented with bilateral periorbital oedema and weight gain. The results of laboratory tests revealed IgAD (IgA < 7 mg/dL), normal creatinine, hypoprotidaemia, hypoalbuminaemia, and nephrotic proteinuria. A diagnosis of IgAD and idiopathic nephrotic syndrome was made, and steroid treatment (prednisone 60 mg/mq/day) was started. During steroid tapering, the children experienced several relapses and to obtain a positive outcome they required therapy with human monoclonal anti-CD20 antibodies (rituximab in the first child, ofatumumab in the second one). Conclusions : Our cases highlight that IgAD can be observed in nephrotic syndrome and nephropathy in children with IgAD appears to be complicated and difficult to treat with corticosteroids alone. Further research is needed to better describe the clinical manifestations and pathological pictures among subjects with IgAD and nephrotic syndrome to understand whether IgAD has a prognostic value in children with nephrotic syndrome and to let clinical physicians define a more personalized and appropriate approach for the management of these patients.

Keywords: IgA deficiency; monoclonal antibody; nephropathy; nephrotic syndrome; pediatric nephrology (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2018
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