The pathogenetic aspects and gene polymorphisms of IgA nephropathy.
Immunoglobulin A nephropathy is an immune-complex-mediated glomerulonephritis characterized by diffuse mesangial deposition of immunoglobulin A or IgA--containing immune complexes. Although its most common clinical presentation is macroscopic hematuria provoked by upper respiratory tract infection, this is neither universal nor necessary for the diagnosis. The patients with IgA nephropathy manifest with variable clinical symptoms (e.g., microhematuria with preserved renal function or progressive deterioration of renal functions resulting in end-stage renal disease). The pathogenetic mechanisms include the abnormality of O-glycosylation of the IgA1 molecule, genetic factors, environmental factors and various inflammatory mediators. The source of mesangial IgA deposits is total circulating serum IgA but the response of the mesangium and the mesangial cells to the deposited IgA is critical to the development of IgAN. Without a genetic predisposition to IgAN, IgA deposition can cause no risk for triggering glomerulonephritis. If generic progression risk factors of an unfavourable outcome coincide (e.g. hypertension, severe proteinuria, elevated serum creatinine level), this will increase the likelihood of progressive renal impairment. Further studies are needed to disclose the precise pathogenetic mechanisms involved in primary IgA nephropathy and to facilitate the development of newer therapeutic possibilities.
Maixnerová
D
D
Department of Nephrology of the First Faculty of Medicine, Charles University and General Teaching Hospital, U Nemocnice 2, 128 08 Prague 2, Czech Republic. dkment@centrum.cz
Merta
M
M
Reiterová
J
J
Stekrová
J
J
Rysavá
R
R
Obeidová
H
H
Tesar
V
V
D005922:Glomerulonephritis, IGA / Q000235:genetics* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D011110:Polymorphism, Genetic