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Diagnosis: IgA Nephropathy
IgA nephropathy occurs in persons of all ages but is still most common in the second and third decades of life. IgA nephropathy is uncommon in children younger than 10 years. In fact, 80% of patients are between the ages of 16-35 years at the time of renal biopsy (Ilyas M.Pediatric IgA Nephropath. In: eMedicina [Link] Visited on July 27th, 2018).
The clinical presentation of IgAN varies across the spectrum of initial renal manifestations, ranging from microscopic hematuria to end-stage renal disease (ESRD). The prognosis was initially considered to be benign in children but long-term studies have shown that IgAN in children can follow the same progression as in adults. Furthermore, previous studies on this subject have shown that ESRD will occur in 6%–43% of patients with IgA nephropathy over a period of 10 years. (Bulut IK, et al. Outcome results in children with IgA nephropathy: a single center experience. Int J Nephrol Renovasc Dis. 2012;5:23-8 [PubMed link]) .
Three things are very interesting in our case: 1: Evidence of chronic changes in the biopsy despite the patient's short age and the apparent short time of evolution of the symptoms; 2: The lobed pattern with double contours that would suggest a membranoproliferative GN; and 3: The subendothelial deposits evidenced in the IF. All this highlighting the tremendous variability in the clinical and morphological expression of IgAN. Perhaps IgAN is not a single disease, but different alterations with a common immunopathological expression: predominant IgA deposits in glomeruli.
In a study published in July 2018, the authors found that the presence of subendothelial deposits of IgA was associated with a significantly increased risk for end-stage renal disease, transplant, death and/or doubling of SCr. (Alvarado AS, et al. Location of glomerular immune deposits, not codeposition of immunoglobulin G, influences definitive renal outcomes in immunoglobulin A nephropathy. Nephrol Dial Transplant. 2018;33(7):1168-1175. [PubMed link]).
See the chapter: IgA Nephropathy of our Tutorial.
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References
- Alvarado AS, Andeen NK, Brodsky S, Hinton A, Nadasdy T, Alpers CE, Blosser C, Najafian B, Rovin BH. Location of glomerular immune deposits, not codeposition of immunoglobulin G, influences definitive renal outcomes in immunoglobulin A nephropathy. Nephrol Dial Transplant. 2018;33(7):1168-1175. [PubMed link]
- Cambier A, Rabant M, Peuchmaur M, Hertig A, Deschenes G, Couchoud C, Kolko A, Salomon R, Hogan J, Robert T. Immunosuppressive Treatment in Children With IgA Nephropathy and the Clinical Value of Podocytopathic Features. Kidney Int Rep. 2018;3(4):916-925. [PubMed link]
- Fabiano RCG, Araújo SA, Bambirra EA, Oliveira EA, Simões E Silva AC, Pinheiro SVB. The Oxford Classification predictors of chronic kidney disease in pediatric patients with IgA nephropathy. J Pediatr (Rio J). 2017;93(4):389-397. [PubMed link]
- Mizerska-Wasiak M, Turczyn A, Such A, Cichoń-Kawa K, Małdyk J, Miklaszewska M, Pietrzyk J, Rybi-Szumińska A, Wasilewska A, Firszt-Adamczyk A, Stankiewicz R, Szczepańska M, Bieniaś B, Zajączkowska M, Pukajło-Marczyk A, Zwolińska D, Siniewicz-Luzeńczyk K, Tkaczyk M, Gadomska-Prokop K, Grenda R, Demkow U, Pańczyk-Tomaszewska M. IgA Nephropathy in Children: A Multicenter Study in Poland. Adv Exp Med Biol. 2016;952:75-84. [PubMed link]
- Mizerska-Wasiak M, Małdyk J, Turczyn A, Cichoń-Kawa K, Rybi-Szumińska A, Wasilewska A, Bieniaś B, Zajączkowska M, Miklaszewska M, Pietrzyk J, Demkow U, Roszkowska-Blaim M, Pańczyk-Tomaszewska M. Predictors of Progression in IgA Nephropathy in Childhood. Adv Exp Med Biol. 2017;955:65-73. [PubMed link]
- Shima Y, Nakanishi K, Sato M, Hama T, Mukaiyama H, Togawa H, Tanaka R, Nozu K, Sako M, Iijima K, Suzuki H, Yoshikawa N. IgA nephropathy with presentation of nephrotic syndrome at onset in children. Pediatr Nephrol. 2017;32(3):457-465. [PubMed link]