IgA Nephropathy

A. Definition
• Immunoglobulin A (N-IgA) nephropathy is a primary
glomerulonephritis disease due to an immune
complex process that is typically characterized by
histopathological features of IgA deposits in the
glomerular mesangial area through
immunohistochemistry and immunofluoresens.
B. Epidemiology
• Nephropathy-IgA disease was first reported by
Berger and Hinglais in 1960. N-IgA events are
frequently found throughout the world and around
20% of IgA particles are found from kidney biopsy
specimens in about 1.6% of potential kidney donors
in Japan.
• 40% of IgA nephropathy progresses from clinical
progression to end-stage kidney disease (PGTA).
C. Pathogenesis and
Pathophysiology
There are 3 important things that are key to the pathogenesis of IgA
nephropathy, namely:
• Abnormal glycosylation of IgA forms the IgAI polymeric molecule
as the nucleus of N-lgA pathogenesis
• Reduced activity of the B3-galactosyl transferase enzyme in B
cells which interferes with the regulation of the immune system
where abnormal glycosylation of IgA is a stimulus for
excessivesialyl transferase
• Galactose 0-glikan enzyme deficiency in the hinge region causes
the formation of excessive polymeric IgA, then forms immune
complexes (IgA-IC) called Hing1 and IgAI molecules that bind to
IgA antibodies (IgA-IgG) called Hing2 which independently cause
damage to the glomerulus.
D. Diagnosis
The various clinical manifestations of N-IgA can be:
• Microscopic haematuria recurs around 40-50%, often preceded
by upper respiratory tract infections so it is called symptomatic
hematuria. This episode is triggered by tonsillitis or upper
respiratory tract infections. In acute circumstances patients often
complain of low back pain.
• Hematuria with proteinuria around 20-40%, while gross
hematuria around 20-25%
• Nephrotic syndrome picture; Rapidly progressive acute
glomerulonephritis characterized by edema, hypertension,
hematuria with renal insufficiency occurs in about 10% of cases.
• Chronic kidney disease N-IgA
The diagnosis of IgA nephropathy is:
• Renal biopsy is the key to N-IgA diagnosis, namely
the discovery of IgA deposits on
immunofluorescent or immunoperoxidase
examination
• Blood IgA levels increase but do not reflect the
severity of the disease
E. Therapy
• Angiotensin converting enzyme inhibitors (ACE-i) or
angiotensin II receptor antagonists (angiotensin II
receptor antagonists, AIIRA / Angiotensin Receptor
Blockers, ARBs).
• Fish oil
• Statins
• Anti-platelet / anticoagulant
• Tonsillectomy plus steroid pulse dose (TSP)
• Immunosuppressive therapy