See u in 3m ē new #pedneph topic
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Sincerely:
Shweta Shah M.D; FASN
Sudha Mannemuddhu, MD, FAAP, FASN, FNKF
Swasti Chaturvedi
M Abdul Qader🇧🇩
Franklin Loachamin
Celeste

#IPNAJC

What is your take-home message?

❓Will you change your induction regimen for LN based on this paper?
❓How can we do better as pediatric nephrologists to achieve early complete remission in LN?
❓Is there more we can do to obtain an earlier diagnosis of LN?
#IPNAJC

X3E
🔭Predictive factors⏩Long-term remission
🎯In Children & adults
👉eGFR @ onset
👉LN class
👉Nephrotic proteinuria

Euro-LN Trial👉Improvement in renal impairment & proteinuria (1g/day) @ 6m (Best predictor of long-term kidney outcome)
pubmed.ncbi.nlm.nih.gov/19155235/
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X3D
What about inflammatory markers for prognostication?
🎯Higher dsDNA at diagnosis ē more stable remission, likely due to more aggressive treatment and follow up?

🎯Low C3/C4 at diagnosis associated ē proliferative lesions on biopsy
pubmed.ncbi.nlm.nih.gov/36688943/
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X3C
🔑Findings:
🎯Mean age at onset: 11y 9m [72.8%👧]
🎯At 24m F/U
👉57% CR
👉34% PR
👉89 of 351 stable CR at 6-24m F/U

🎯eGFR ≥90 ml/min @ diagnosis & biopsy class III👉 Predictive of kidney remission

⛔️difference ~ MMF & Cyclophosphamide (maintain stable remission)
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X3B
In both adult & pediatric studies, MMF was found as effective as IV cyclophosphamide in inducing remission.

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X3A
🎯Complete remission (CR) at 24m F/U was in 52.9% (of 382)⏭️This is greater than earlier studies (38% at 12m by Suhlrie et al)
pubmed.ncbi.nlm.nih.gov/32193650/

Adult studies👉shown those who have achieved either partial or CR at 24m had a ⏬Risk of future renal failure or mortality

Welcome Celeste
#IPNAJC

X2O
❓What about renal transplantation in this group of patients?
👉Two patients received renal transplantation during 2 years follow-up
👉One received after renal transplantation at 4 years follow -up
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X2N
🎯What about the use of renal replacement therapy in this group of patients?
👉9 patients required renal replacement therapy
👉6 were on hemodialysis

⏭️AKI was the reason for dialysis among 6/9 patients
⏩5 could stop dialysis after renal recovery
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X2M
In Subgroup (351 patients ē available data)
👉25.4% maintain stable complete remission (CR) from 6-24m F/U

Reasons for⛔️Stable CR
⏩Low eGFR (12.6-20.9%)
⏩Proteinuria (19.8-33.8%)
⏩Both (6.5-9.9%)

What patient characteristics you observe in difficult-to-treat LN?
#IPNAJC

X2L
👀👀Let’s look at the patients’ outcomes

🗓️At 6 months,
👉Half of the children achieved complete remission
👉A further third had partial remission

🎯Patients with class III LN more often had complete remission at 2 years compared to others.
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X2K
🎯Steroid withdrawal 29.1% (Short F/U) & 37.9% (Long F/U)
🎯Higher % of child on ARBs (Long F/U)

🗓️After 12m
👉higher anti-dsDNA in MMF group [Med 25IU/ml (8.1-100.5)] vs Cyclophosphamide [Med 13 IU/ml (0-50)]

❓Do you withdraw steroids
❓Are there specific groups
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X2J
⏭️Maintenance include:
👉Oral steroids 90%
👉MMF 51.8%
👉Azathioprine 13.4%
A few👉Tacrolimus, Cyclosporine, Methotrexate & Mizoribine <3%

⏩Median dose
👉MMF 32.3 (24.4-39.4) mg/kg/d
👉PNL 0.4 (0.2-0.6) mg/kg/d

❓What dose of PNL your institute use for maintenance?
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X2J
Let’s look at the graphical representation of induction and maintenance therapy in these 382 patients

X2I
🗓️How long will you wait before starting anti-proteinuric treatment for patients who have renal impairment on their presentation❓

👉Will you wait for complete normalization of their creatinine?

If so, how long will you wait for❓❓
#IPNAJC

X2H
Let’s look at anti-proteinuric treatment
🎯51.8% of children were treated ē either
👉Angiotensin-converting enzyme inhibitor (ACEi) or
👉Angiotensin receptor II blocker (ARB)

🎯A higher percentage of children received ACEi (p=0.003) among children <12y & 4m

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X2G
What about steroids use for induction?
💉IV Methylprednisolone (70.9%)
💊Oral Prednisolone (95%)

Child <12y & 4m👉Higher doses of steroid (IV MP & oral PNL) (p= <0.0001)
Older child 🟰 higher doses of Rituximab (p=0.03)

Do you use more steroids in young population?
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X2F
👀Let us now look at the therapy regimens used for induction.

Most common drugs used for induction
💊Mycophenolate mofetil (MMF) (45.5%)
💉IV Cyclophosphamide (50%)
💊Azathioprine
💉Rituximab

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X2E
🎯Females had higher anti-dsDNA
🎯Children older than 12y & 4m had higher Anti-dsDNA

👉eGFR were lower in patients with higher class LN (Class IV & Class V mixed with Class III & IV)

#IPNAJC