IgA nephropathy

IgA nephropathy is the most prevalent primary chronic glomerular disease worldwide.

Diagnostic manifestation is the predominance of the IgA deposits either alone or with IgG, IgM, or both in the glomerular mesangium.

The frequency of IgA without IgG or IgM ranges from zero to more than 85%, depending on the center.

C3 and properdin are almost always found, while C4 or C 4d, mannose binding lectin and complement C5-9 are frequently noted while C1q is absent.

Alternative and lectin pathways of complement activation are possibly involved in the pathogenesis.

On light microscopy there is an increase in mesangial matrix and hypercellularity.

Light microscopy may also show focal necrosis, segmental scarring and crescents in Bowman’s space.

Electron microscopy shows immune deposits generally in the mesangial and paramesangial areas of the BM.

Has similar renal histologic features of Henoch-Shonlein purpura nephritis.

About 75% of children and young adults present with macroscopic hematuria during an upper respiratory or gastrointestinal tract illness.

Patients may have evidence of acute kidney injury.

Adult patients usually have proteinuria, microscopic hematuria, hypertension, alone or in combination.

In the United States more than 50% of all those older than 30 years of chronic kidney disease stage III-five.

Male to female ratio is 2:1 for children and adults.

In Asia the male: female ratio is 1:1.

The nephrotic syndrome is uncommon presentation.

Studies support the use of immunosuppressive therapy to target mesangial IgA deposits and circulating autoantibodies.

Guidelines recommend treatment with a blocker of the renin-angiotensin system in patients to have proteinuria with urinary protein excretion of more than one gram per day.

Treatment guidelines also suggest the use of systemic glucocorticoids in patients who have proteinuria of greater than one gram of urinary excretion per day in the GFR higher than 50 mL per minute despite supportive care.

In a randomized controlled trial of supportive care versus supportive care and immunosuppressive therapy over 3 years in patients with high-risk IgA nephropathy: there was no significant improvement in outcome but adverse effects were observed more frequently in those who received the immunosuppressive therapy with no change in the rate or decrease in the glomerular filtration rate (Rauen T et al).

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