340.4 📋 章末速記 Summary

340.4.1 🔑 一句話總結

Glomerular diseases 細目 (specific GN treatments)：(1) MCD — pediatric NS 80%, podocyte foot process effacement only on EM, steroid-responsive 80-90%; adult MCD think Hodgkin, NSAIDs, lithium；(2) FSGS — adult US most common, variants (NOS, perihilar, tip, cellular, collapsing worst — HIV/COVID/APOL1); primary IS; secondary treat underlying; sparsentan DUPLEX 2023 FDA approved；(3) MN — older adults, anti-PLA2R 70-80% diagnostic + monitoring; subepithelial deposits + GBM spikes; rituximab first-line (MENTOR 2019); renal vein thrombosis risk (anticoagulate if albumin < 2.0-2.5)；(4) IgA nephropathy — globally most common GN; synpharyngitic gross hematuria classic; mesangial IgA; MEST-C scoring; Nefecon (targeted-release budesonide FDA 2021) + sparsentan PROTECT 2023 + iptacopan APPLAUSE emerging；(5) lupus nephritis — ISN/RPS Class I-VI; Class IV most aggressive; MMF + steroids induction; voclosporin AURORA 2020 + belimumab BLISS-LN 2020 added for refractory；(6) ANCA-associated vasculitis (GPA + MPA + EGPA) — pauci-immune crescentic GN; rituximab + steroids (PEXIVAS); avacopan FDA 2021 adjunct；(7) anti-GBM (Goodpasture) — linear IgG, pulmonary-renal; plasmapheresis + cyclophosphamide + steroids emergency；(8) MPGN / C3G — immune complex (lupus, hep C, monoclonal) or complement-mediated (DDD, C3GN); iptacopan trials；(9) DKD — globally most common CKD/ESKD cause; Kimmelstiel-Wilson nodules; 4 pillars therapy；(10) AL amyloid — myeloma-associated; daratumumab + CyBorD ANDROMEDA；2024 emerging: inaxaplin for APOL1 nephropathy。

340.4.2 💊 治療精要

• MCD：prednisone 1 mg/kg × 4-8 wk → taper；relapsers MMF, rituximab, CNI
• FSGS primary：prednisone 1 mg/kg × 4 mo；resistant → cyclophosphamide, MMF, rituximab, CNI；sparsentan DUPLEX 2023
• MN：rituximab (MENTOR 2019) first-line OR Ponticelli regimen (cyclophosphamide + steroids alternating) OR CNI；anti-PLA2R monitoring + anticoagulation if albumin < 2.0-2.5
• IgA：ACE/ARB + SGLT2i + Nefecon (targeted-release budesonide FDA 2021) + steroids if persistent + sparsentan PROTECT + iptacopan APPLAUSE-IgAN
• lupus nephritis Class III/IV：MMF (induction) or cyclophosphamide + steroids + voclosporin (AURORA 2020) added + belimumab (BLISS-LN 2020) + maintenance MMF/AZA + hydroxychloroquine all SLE
• ANCA vasculitis：rituximab + high-dose steroids (PEXIVAS, RAVE, RITUXVAS) OR cyclophosphamide + avacopan (FDA 2021 adjunct) + plasmapheresis for severe (alveolar hemorrhage)
• anti-GBM：plasmapheresis daily 14-21 d + cyclophosphamide + steroids emergency ± rituximab
• MPGN/C3G：treat underlying + IS; iptacopan (factor B inhibitor) APPLAUSE for select + eculizumab for terminal complement
• DKD：4 pillars (ACE/ARB + SGLT2i + finerenone + GLP-1 RA) — see Ch335
• AL amyloid：Dara-VCD (daratumumab + bortezomib + cyclophosphamide + dexamethasone) ANDROMEDA 2021 front-line

340.4.3 🎯 盧醫師的考前提醒

1. MCD podocyte foot process effacement only on EM；LM 正常；IF negative；adult MCD 鑑別 Hodgkin / NSAID / lithium
2. FSGS variants Columbia：NOS, perihilar, tip, cellular, collapsing (worst, HIV/COVID/APOL1 risk)；APOL1 G1/G2 in African ancestry ↑ risk
3. MN anti-PLA2R 70-80% primary + diagnostic + monitoring；anti-THSD7A 3-5%；anti-NELL-1 associated with malignancy in older patients
4. MENTOR (2019) rituximab > cyclosporine for primary MN — now first-line；Ponticelli regimen alternating cyclophosphamide + steroids historical
5. IgA nephropathy features：synpharyngitic gross hematuria within 1-2 days of URI；mesangial IgA deposits；MEST-C Oxford scoring + Nefecon (Tarpeyo) FDA 2021
6. lupus nephritis ISN/RPS Class I-VI (2003)；Class IV (diffuse proliferative ≥ 50% glomeruli) most aggressive；Class V membranous；Class III/V or IV/V combinations common
7. BLISS-LN (2020) belimumab + AURORA (2020) voclosporin — recent additions to lupus nephritis armamentarium；BAFF inhibitor + new CNI respectively
8. ANCA-associated vasculitis 3 syndromes：GPA (PR3, c-ANCA — ENT + lung + kidney)、MPA (MPO, p-ANCA — kidney + lung)、EGPA (MPO mostly — asthma + eosinophilia + neuropathy)
9. avacopan (Tavneos) FDA 2021 for ANCA vasculitis：C5a receptor antagonist + steroid-sparing；adjunct to standard
10. anti-GBM (Goodpasture) emergency：pulmonary-renal syndrome + linear IgG along GBM + crescents；plasmapheresis daily 14-21 days + cyclophosphamide + steroids — time-sensitive