Q.778. Enumerate the causes of recurrent diseases post transplantation?
﴾﴾ VI. Recurrence ﴿﴿
 |
.Renal transplantation- Allograft |
Q.778. Enumerate the causes of recurrent diseases post transplantation?
A. Causes
of recurrent diseases post transplantation:
1) Idiopathic FSGS ➤ 40%
2) M.N.➤ 40 %.
3) M.P.➤ type I.: 25 % & type II.: 80
%
4) IgA Np.: ➤ 25%
5) TTP/HUS ➤ ClassicØ1 %, atypical➤20%, familial:80%.
6) Anti-GBM A.B.: ➤ as high as 50%.
7) PHx. (Oxalosis): (80-100)
%.
8) Diabetic Np.: (80-100) %.
9) S .L.E.: (3-10) %.
10) Wegener’s
dis.: < 20 %.
11) Fabry dis.:
< 5 %.
Q.779. How frequent is FSGS recurrence after T.x.?
How to manage?
A. Incidence
of recurrence of Iry FSGS reaches: 85 % (children) & 40 % (adults). Rapid
recurrence occ. é👉Circulating G. permeability f.. Other causes incl.:
[variety of viruses
& drugs, incl.: Siros, podocin mutations (rare)]. Also described
Presentation: [Rapid onset of NRP
]. To detect early recurrence: screened
for Prot é
spot URAR é
1st postop. d., day of hospital discharge, then
weekly /4 w.,
then monthly for one y. after Tx.. If
ratio > 0.5 ➤ 24-h.
protein. R. biopsy performed if post-Tx. Prot >1 g./d. & histologic
analysis should incl. E/M. Most immediate finding in recurrent FSGS 👉 foot process effacement,
final Dgx. requires ➤ finding of FSGS
on L.M., similar to Dgx in Iry FSGS.
Optimal
ttt is not known, but the foll. is suggested: Rapid recurrence shd
be ttt é Pph..typical
reg.: 1.5
pl. vol. exchanges/3
consecutive d.s, then E.O.D./total 2 w.s. IV.IG (500 mg/kg) shd be given after Pph.
to replace the I.G. removed by Pph..
Late recurrence: defined
as later thn one
y. after T.x.➤ Csp (100 mg orally d.) as replacement
for the antimetabolite such as Aza or MMF. If no resp.
after 6-12
w., we attempt Pph.,
é same regimen described for rapid
recurrence. ACEI/ARBS should be
initiated unless C.I. & Hyperlipidemia➤ HMGCoA, Homozyg. or heterozygous
podocin mutations ➤ aggressive
thpy
(Poor response).
Q.780. What is the chance of membranous nephropathy to recur after transplantation?
A. MN recurrence
rate:
10-45 %. Mean
time to recurrence, (typically as NRP) = 10 m. Affected ptns hv more aggressive
initial dis. (progression to ESRD é 4 y.). The M-type
phospholipase A2 receptor (PLA2R بلاتور , a transmembrane
rec-eptor highly expressed in G. podocytes: def. as a mj. Ag in
human idiop. MN. Pres. of auto-A.B. agnst this
receptor,👉 underlying
cause of recurrent MN.
Csp, tcrol. & MMf. don’t protect agnst dis.
recurrence & there’s no evidence tht additional
im/m. thpy alters the course. Rituximab successfully ttt recurrent disease➤ partial or complete
remission.. Post.ttt biopsies: “resorption of electron dense” immune
deposits. (due to depletion of
anti PLA2R autoA.B.). Living-related Tx. are at higher
risk for recurrence , compared to deceased donorTx..
Q.781. How frequent is TTP-HUS recurrent after transplantation? How to manage?
A. Recurrence rate of HUS after R.Tx. = 25-50 % (overestimate, since both Ac. vasc. Rj. & CNI
👆 vascular
injury &TMA indistinguishable fr. Iry disease).
Multiple clinical ch. ch. may be ass. é recurrent
HUS :
o
P.H. of non-Drr. causes (non-shiga
associated [NStx] HUS), much higher risk of recurrence
thn those due to Drr.
o
Recurrence
significantly ass.
é :
1.
“Older age” at onset of HUS.
2.
Short duration betw. dis. onset & ESRD or Tx.,
3.
Living related donors
& to a lesser degree, CNI.
4.
Familial HUS, due to mutations é complement inhibitor genes .
5. Limited evidence: lower recurrence rate é “nephrectomy”.
Affected ptn.s typically present with a “microangiopathic
hemolytic anemia”, thrombocytopenia
& ARF. Graft loss reported in 10-50 % of cases.
Prognosis: Overall graft success may be diminished. Recurrence is invariable
é familial dis.. Management: two phases: 1st phase ➤ Prevention
by:
[Low-dose
aspirin + dipyridamole]. [Csp & ALS] shd be used cautiously.
2nd phase: ➤ ttt of recurrent HUS. Csp dose shd be reduced
by 50% é 1st
Sn of recurrence. Pl. infusions & pl. exchange, similar to tht used
for Iry disease
Q.782. How frequent is IgA
nephropathy recurrence after transplantation?
A. Donor
source hs a role in timing of recurrence ('Donor effect'). So,
the adv. in allograft survival é living-related
comp. to deceased donor may be negated.
Ptn.s é rapidly progressive course of native IgAN should
be informed tht recurrence may present early,
wch. should not preclude consideration for subsequent re-T.x.
ACEI or ARBs should
be initiated é evidence of recurrence.
Whether routine, early post-Tx. initiation of these drugs to all recipients é native IgAN, independent of
recurence, is beneficial is unknown. At least, they should be incl. as first line
anti-H.T. Benefit of fish oil, remain to be tested. There’s no
evidence that any im/m., alone or in combination, is beneficial in preventing
recurrence or altering the course of recurrence.
Q.783. How likely is anti-GBM antibody disease to recur after transplantation?
A. Incidence of recurrence: as
high as 50 %.
However, most ptn.s ➤ aSm.c.
The relatively low rate of significant recurrence is thought to be due
to delaying R.Tx. until ➤ Circulating
anti-GBM A.B. hv bn undetectable for at least 12 m. & there hs bn 6 m. at least
post-ttt quiescent dis (without
cytotoxic Ag.). Lack of late recur-ence
reflects the self-limited nature of autoA.B. formation in this
dis.. In addition, maintenance im/m. therapy➤ may
help suppress autoA.B. production.
Clinically evident recurrence
typically present é[hematuria & proteinuria].“Spontaneous
resolution” can occur Graft loss due to recurrent anti-GBM A.B. dis. is rare.
ttt.:[Pulse steroids, Cph.
& pl. exchange] , esp é life-threatening pulm. disease.
Q.784. How likely is Membranoproliferative G.N. to recur after transplantation?
A. Recurrent
MPGN is not uncommon event,
esp. é type II disease.While expected graft survival is dcr. é setting of recurrent MPGN, many
grafts will continue to hv adequate function & eventually lost
for reasons other than recurrence.
Given the lack of a proven beneficial
therapeutic intervention, aggressiveness of ttt. should match the aggressiveness
of disease process. Presence of hepatitis C & CMV should be evaluated & ttted accordingly. No
data suggest: T.x. é deceased
donor rather a living donor hs favorable effect on graft
survival by decrease likelihood of recurrence.
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