[Immunity and tubular dysfunction in case of systemic disease]

Abstract

The immune renal tubular diseases are known since five decades, but their prevalence remains to be defined. They are caused by humoral and cellular effectors of innate and adaptative immunities on several targets of the renal tubule: protein channels, co or counter transporters, luminal or cytosolic enzymes, tight junctions. Genetic or epigenetic variations are also involved. Clinical manifestations are various and make the diagnosis difficult. They can precede the causal affection and they worsen the prognosis. The classical model consists in hypokalemic tubular distal acidosis observed in Sjögren's syndrome which illustrates the auto-immune epithelitis concept. Cellular immunity can act through other ways, like tertiary lymphoid neogenesis in systemic lupus. Humoral immunity through autoantibodies targets several membrane, cytosolic or nuclear proteins, causing specific tubular dysfonctions. It is also implied in the epithelial-mesenchymal transition of tubular cells. Innate immunity through cytokines may be involved. Treatment consists in electrolytic disorders correction and immunosupppressive medication: the choice should be guided at best by physiopathology.

Keywords: Acidose tubulaire distale hypokaliémique; Adaptative immunity; Auto-immune epithelitis; Cellule tubulaire proximale; Hypokalemic distal tubular acidosis; Immunité adaptative; Immunité innée; Innate imunity; Néogenèse lymphoïde tertiaire; Proximal tubular cell; Tertiary lymphoïd neogenesis.