ACR 2024 - Day 3 Report Save

Highlights from Day 3 at ACR24 included the plenary session presentation on Nipocalimab (previously reviewed), but the big highlight was the Lupus Nephritis guideline recommendations.

2024 Updated ACR Guideline for the Diagnosis and Treatment of Lupus Nephritis

• Session 18M17 – This session featured presentations by Drs. Sammaritano, Dall’Era, Askanase, and Son. They presented the work of the Guideline committee that had the stated objective to preserve renal function and reduce morbidity, collaborate with nephrology, encourage shared decision-making, and address pediatric cases as well.  Overall they put forth 28 graded recommendations – 7 Strong, 21 Conditional and 13 ungraded good practice statements (GPS).  The guidelines are available from the ACR website  
• Below are some of the highlights of these guidelines:
  • Screening: SLE (without kidney disease) strongly recommend screening for proteinuria at least every 6-12 months, OR when experiencing extra-renal flares.
  • Kidney Biopsy: kidney biopsy should be done in SLE when LN is suspected.
    • Conditionally recommend kidney biopsy in SLE with proteinuria >0.5 g/g and/or impaired kidney function not otherwise explained.
      • Also, in treated LN in remission presenting with suspected LN flare (increased proteinuria, hematuria, and/or worsening kidney function)
  • Treatment of Active LN (CLASS III/IV OR CLASS V)
    • GPS: Prompt glucocorticoid treatment (before kidney biopsy)
    • GPS: Dosage of LN medications should be adjusted.
    • GPS: Adjunctive systemic anticoagulation in LN with significant risk factors for thrombosis (e.g., low serum albumin in context of severe proteinuria)
    • All should be on hydroxychloroquine.
    • SLE with proteinuria, should be on RAAS-I
    • Recommend pulse intravenous glucocorticoids 250-1000 mg methylprednisolone daily x 1- 3 days, followed by oral glucocorticoid ≤0.5 mg/kg/day (maximum dose 40 mg/day) with taper to a target dose of ≤5mg/day by 6 months.
    • Treatment with any triple immunosuppressive therapy for at least 3-5 years. Triple therapy includes steroids plus 2 more agents.
      • MPAA (mycophenolate) plus belimumab -or
      • MPAA plus CNI -or
      • Euro Lupus Nephritis Trial (ELNT) low-dose CYC plus belimumab
    • Conditionally recommend an MPAA-based regimen over a CYC-based regimen
    • If proteinuria ≥3g/g, we conditionally recommend a TRIPLE immunosuppressive regimen that contains MPAA plus CNI.
    • With extra-renal manifestations, we conditionally recommend a TRIPLE immunosuppressive regimen that contains belimumab.
  • Treatment of Active LN (pure CLASS V)
    • If proteinuria ≥1 g/g : conditionally recommend TRIPLE immunosuppressive regimen of glucocorticoids with MPAA plus CNI.
    • If proteinuria < 1g/g: conditionally recommend glucocorticoids and/or immunosuppressant therapy (MPAA, AZA, or CNI).
  • Treatment of Nonresponsive or Refractory LN
    • GPS: Medication dose and patient adherence should be assessed.
    • GPS: With an inadequate renal response: conditionally recommend escalation of treatment: 
      • For initial DUAL therapy, escalate to TRIPLE therapy.
      • For initial TRIPLE therapy, change to an alternative TRIPLE therapy or consider addition of an antiCD20 agent as a second immunosuppressive, or combination therapy with three non-glucocorticoid immunosuppressives (i.e., MPAA, belimumab and CNI), or referral for investigational therapy.

Subcutaneous Ianalumab (VAY736) in SLE – a Phase 2

• Abstract 2580 – presented by Mysler, 68 SLE patients were treated with either VAY or placebo for 28 weeks. Then placebo patients rolled over to active VAY treatment and patients were followed out to week 68. VAY was shown to be highly effective at depleting B cells, improving serologic measures and showed significant efficacy (compared to placebo) in multiple SLE outcomes – including SRI-4, SRI-6, LLDAS and DORIS.