The immunopathogenesis of idiopathic nephrotic syndrome: a narrative
review of the literature
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Abstract
Idiopathic nephrotic syndrome (INS) is a common glomerular disease in
childhood, and the immunological involvement in the pathogenesis of
non-genetic INS, although not fully elucidated, is evident. This
narrative review aims to offer a concise and in-depth view of the
current knowledge on the immunological mechanisms of the development of
INS as well as the role of the immunological components of the disease
in the responsiveness to treatment. T cell immunity appears to play a
major role in the INS immunopathogenesis and has been the first to be
linked to the disease. Various T cell immunophenotypes are implicated in
INS, including T-helper-1, T-helper-2, T-helper-17, and T regulatory
cells, and various cytokines have been proposed as surrogate biomarkers
of the disease; however, no distinct T helper or cytokine profile has
been conclusively linked to the disease. More recently, the recognition
of the role of B cell mediated immunity and the various B cell subsets
that are dysregulated in patients with INS have led to new hypotheses on
the underlying immunological causes of INS. Finally, the disambiguation
of the exact mechanisms of the INS development in the future may be the
key to the development of more targeted personalized approaches in
managing INS.
Conclusions: INS demonstrates particularly interesting
immunopathogenetic pathways, in which multiple interactions between T
cell and B cell immunity and the podocyte are involved. The
disambiguation of these pathways will provide promising novel
therapeutic targets in INS.
What is Known:
INS is the most common glomerular disease in the paediatric population,
and its onset and relapses have been linked to various immunological
triggers.
Multiple immunological mechanisms have been implicated in the
pathogenesis of INS; however, no single distinct immunological profile
has been recognized.
What is New:
Th17 cells and Treg cells play an important role in the immune
dysregulation in INS.
Transitional B cell levels as well as the transitional/memory B cell
ratio have been correlated to nephrotic relapses and have been proposed
as biomarkers of INS relapses in SSNS patients