[MIM 161 950]

(IgA nephropathy)

Prevalence: around 1 % of the world's population. Autosomal dominant transmission with variable penetrance of a mutation of the IGAN1 gene ( 6q22-q33) in the majority of the cases. Other cases are caused by a mutation of the IGAN2 gene (2q36) or of the SPRY2 gene (13q31).

 Nephropathy secondary to mesangial IgA deposition associated with different degrees of mesangial proliferation of unknown pathogenesis. Structural abnormalities of the O-glycolysation of the IgA transition region play an essential role in mesangial deposition. 

Microscopic hematuria, often associated with proteinuria and episodes of macroscopic hematuria. Evolution to hypertensive chronic renal failure in 30 % of cases.

Anesthetic implications: 

assessment of renal function; avoid nephrotoxic agents;  NSAIDs are contraindicated.

Updated: November 2019