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summary of EULAR 2019 guidelines SLE Mx.pptx
- 1. EULAR 2019 UPDATEFOR SLE MANAGEMENT DR MOHAMMAD SUHAIL
- 2. 1. Guidelines a) Goals b)Treatment i. HCQ ii. GC iii. Immunomodulators iv. Biologics c) Specific organ involvement 2. Summary of changes compared to 2008
- 3. GOALS OF THERAPY •Target: low disease activity • SLEDAI <= 4 • SLEDAI <=3 on Antimalarials • PGA<=1 with Glucocorticoids <7.5mg/d PREDNISOLONE +- well tolerated immunosuppessants
- 7. GOALS OF THERAPY •Target: Remission or at least low disease activity • SLEDAI <= 4 • SLEDAI <=3 on Antimalarials • PGA<=1 with Glucocorticoids <7.5mg/d PREDNISOLONE +- well tolerated immunosuppessants • In Lupus nephritis: • >50% reduction in proteinuria and S.Cr within 10% from baseline in 12 months • (Complete renal remission: Proteinuria <500mg/d, S.Cr witin 10% baseline) • Prevention of flares • Measurable increase in disease activity that leads to change of treatment
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- 9. HCQ • Recommended toALL patients with SLE (if not contraindicated) • Dose: <5mg/kg/d BW in 2 divided doses • Ophthalmology screening: • At initiation • After first 5 years • Yearly thereafter • QUINACRINE may be considered in HCQ-induced retinal toxicity
- 10. GLUCOCORTICOIDS (GC) • Rapidsymptom relief • Dose <7.5mg/d PREDNISONE EQUIVALENT • 2 strategies to reduce maintenance dose: • Pulse GC (250-1000mg/d MP x 3-5 days) • Early initiation of Immunosuppressant (IS)
- 12. IMMUNOSUPPRESSANTS (IS) • Facilitatesrapid GC tapering, lower maintenance GC • METHOTREXATE (MTX): Strongest supporting data. • Azathioprine (AZA): Published evidence less than MTX but compatible with pregnancy • Mycophenolate Mophetil (Mmf): Shown in recent RCT to be more efficacious than AZA in controlling flares and achieving remission (But not neuropsychiatric involvement). Teratogenic; needs to be stopped 6 weeks before planning pregnancy. • Cyclophosphamide (CYC): considered in organ-threatening disease (Renal, neuropsychiatric,cardiopulmonary involvement). Gonadotoxic. Low dose GnRh, cryopreservation.
- 13. BIOLOGICS • BELIMUMAB: Extrarenaldisease with inadequate control – high disease activity/inability to taper steroids. High disease activity (SLEDAI>10), MSK, serological, cutaneous manifestations show most response. • RITUXIMAB (RTX): Off label. Severe renal/extrarenal not responding to HCQ+GC+IS. More than 1 IS needs to have failed to warrant use of RTX- except severe thrombocytopenia, hemolytic anemia.
- 15. SYSTEMIC DISEASE: SKIN(CLE) • First line: Topic GC+HCQ +/-systemic GC • MTX • BELIMUMAB • RTX • Others: DAPSONE, THALIDOMIDE, EC- Mycophenolic acid • SKIN PROTECTION- broad spectrum sunscreen, smoking cessation • Refractory: skin biopsy
- 16. NEUROPSYCHIATRIC LE (NPSLE) •Effort to link manifestations to SLE – Neuroimaging, etc. • Underlying pathophysiology • Inflammatory- GC, IS • Ischemic/thromboembolic- Anticoagulants. *aPL • CVA with SLE – 1st line SLE management + stroke treatment as in general population • Symptomatic psychiatric treatment
- 17. HEMATOLOGICAL DISEASE THROMBOCYTOPENIA GC +IS Pulse GC AZA, Mmf, CYCLOSPORINE IvIg RTX, CYC Thro-poietin, Splenectomy AIHA GC, IS RTX
- 18. LUPUS NEPHRITIS (LN) •INDUCTION PHASE: • MMF +/- CYCLOPHOSPHAMIDE • MMF+CYC if high risk of progression to ESRD (reduced GFR, HPE – interstitial fibrosis, Tubular atrophy, fibrous crescents/fibrinoid necrosis) • MAINTENANCE: • MMF > AZA • REFRACTORY/RELAPSING CASES: • RTX • Calcineurin inhibitors (CNI)-TACROLIMUS, etc. • F/U • Upr <1g/d at 6m, <0.8g/d at 12 months = good prognosis. Hematuria not considered
- 19. SUMMARY OF CHANGES •Use of Scores to guide drug therapy. • More HCQ, less steroids. HCQ recommended to all patients. Upper limit set to maintenance steroid dose/day • Early initiation of IS, recommendation of GnRH with CYC • BELIMUMAB in extrarenal disease. • Use of proteinuria and not hematuria in F/U of LN
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Editor's Notes
- #3 Complete remission is unlikelySLEDAI 0 with no GC or HCQ
- #7 Complete remission is unlikely;
- #12 MTX Dose 10-25mg/week with folic acid supplementation. Adverse effects: GI intolerance, mucositis, hair loss, hepatotoxicity, myelotoxicity. AZA dose: 1.5-2mg/kg/d in 2 doses initially; may be lowered in maintenance. MMF: mild/mod/maintenance: 1-2g/day in 2 doses. Induction/severe: 3g/day in 2 doses CYC:
- #13 IgG1-Lambda Mab that blocks binding soluble human B-lymph stimulator (BLyS) to R on B-Lymph- reduces B-lymph survival. BELI: IV: 10 mg/Kg on weeks 0, 2, 4, then every 4 weeks ; SC: 200 mg weekly LUNAR trial showed that in LN, B-Lymph depletion with RTX improve chances for complete response at 78 weeks. RTX : 1000 mg on days 1 and 15 - re-administration every 6 months or “on-demand”
- #16 Distinction between the 2 may not be easy; both may be present together – both treatment can be given together
- #17 Cyclosporine dose: 1-3 mg/Kg/day or 100-400 mg/day in 2 doses
- #18 High risk: Juveline onset, Males, anti-C1q Ab positive MMF: 3g/d 2 doses. CYC: 500mg IV alt weeks till 10 weeks (Euro-lupus; LN); 0.75-1g/BSA m2/month x 6 months (NIH; organ or life threatening)