1. IgA nephropathy
2014년 8월 6일
R1 황규환

2. IgA nephropathy m/c cause of primary GN in Developed countries
Peak incidence in Second & Third decades of life
Greatest frequency in Asians & Caucasians
Rare in Blacks
IgA deposits may also be seen in healthy individuals from 3 to 16 percent
(renal allograft donors in Japan, non-selected autopsy in Ger many)

3. Light microscope
focal (involving less than 50 percent of glomeruli) or more often diffuse mesangial proliferation and matrix expansion

4. Immunofluorescence microscope
large, globular mesangial IgA deposits that are diagnostic of IgA nephropathy or Henoch-Schönlein purpura

5. Electron microscope
electron-dense deposits that are limited to the mesangial regions (D). The glomerular basement membrane (GBM) is normal, and there are no glomerular capillary wall deposits.

6. Clinical features IgAN typically present in one ot three ways 40~50%
Gross hematuria, following an URI or athletic exertion
30~40%
Microscopic hematuria and mild proteinuria
Incidentally detected
<10%
Nephrotic syd. or RPGN
Edema, HTN, Renal insufficiency
Rarely, Crescentic IgA nephropathy
AKI, Increase of dysmorphic RBC

7. Treatment Non-immunosuppressive Tx. Immunosuppressive Tx.
ACEi or ARB : BP control and slow progression of renal dz
Statin : Lower cardiovascular risk
Fish oil, Tonsillectomy (are Less clear)
Immunosuppressive Tx.
Glucocorticoid alone
Glucocorticoid + Cyclophosphamide followed by azathioprine
Glucocorticoid + azathioprine
Mycophenolate mofetil(MMF)
Calcineurin Inhibitor(Cyclosporine, Tacrolimus)

8. Treatment Isolated hematuria, no or minimal proteinuria, normal GFR
Typically not treated
Should be monitored at 6~12 months intervals
Persistent proteinuria, normal or nearly normal GFR
Non-immunosuppressive Tx.
Nephrotic range proteinuria, 6개월이상 치료에도 지속되 는 단백뇨, serum Cr. ↑, severe histologic findings on Bx.
Immunosuppresive Tx. in addition to Non-immunosuppressive Tx.
Chronically elevated Cr., prominent Glomerulosclerosis, Tubulointerstitial atrophy or fibrosis
Not treating with Immunosuppresive Tx.

9. Non-immunosuppresive tx.
ACEi or ARB
Lowering both systemic BP and intraglomerular pr.
Minimizing proteinuria and slowing rate of progression of renal dz.
Greater efficacy compared with other anti-HTN drug
Combination of ACEi & ARB (?)
Statins
Lower cardiovascular risk
But No Evidence on slowing the progression of renal dz.
Fish oil
Maybe act by anti-inflammation
Randomized trials : report conflicting results

10. Immunosuppresive tx. Glucocorticoid alone
Significantly reduce incidence of Renal events
When ACEi or ARB has failed to lower proteinuria
Glucocorticoid + Cyclophosphamide followed by azathioprine
with more severe Dz (rapidly progressive clinical course or Crescent formation)
PDL(40mg/day, tappered to 10mg/days) 2 years
Cyclophosphamide 3 months -> Azathioprine 2yrs
Glucocorticoid + azathioprine
Severe disease in children
Mycophenolate mofetil(MMF)
Unclear role, Fetal risk↑
Calcineurin Inhibitor(Cyclosporine, Tacrolimus)
No benefit, Relapse

11. Prognosis Clinical predictors
At the time of Dx., a marker for more severe Dz.
Elevated serum Cr.
HTN (140/90mmHg)
Persistent(eg, for more than six months) protein excretion above 1000mg/day
Histologic predictors(Oxford classification of IgAN)
Mesangial hypercellularity
Segmental glomerulosclerosis
Endocapillary hypercellularity
Tubular atrophy/interstitial fibrosis
Serologic predictor
High serum levels of “Poorly galactosylatedd IgA1” : Bad prognosis

12. Recurrence after KT Risk factor Clinical manifestation
Use of living-related donor kidney
Specific HLA alleles in recipient, including HLA-B35, HLA- DR4, HLA-B8, DR3
Good HLA match between donor and recipient
High serum IgA concentration
Clinical manifestation
persistent microscopic hematuria
New or worsening proteinuria
Increase in serum creatinine

13. Recurrence after KT Treatment ACEi or ARB
IgAN & HTN : use ACEi or ARB even in absence of evidence of recurr
Immunosuppressive Tx.
: biopsy-proven recurrence
rapidly rising serum Cr
nephrotic range proteinuria
: high-dose glucocorticoids (PDL 1mg/kg per day) for 2 mo nths
: Cyclophosphamide

14. 감사합니다.