340.2 🩺 國考版

340.2.1 高頻考點

340.2.1.1 MCD (Minimal Change Disease)

• Pediatric NS 80%
• Foot process effacement on EM
• Steroid-responsive
• Selective proteinuria
• Adult: Hodgkin, NSAID, lithium

340.2.1.2 FSGS

• Adult NS US most common
• Variants: NOS, perihilar, tip, cellular, collapsing
• Causes: idiopathic, genetic, secondary (HIV, obesity, reflux)
• APOL1 in African ancestry
• Collapsing variant: worst prognosis
• Treatment: steroids, CNI, rituximab, sparsentan emerging

340.2.1.3 MN

• Older adults (60+)
• Anti-PLA2R 70-80% primary
• Anti-THSD7A, anti-NELL-1 less common
• Secondary: hep B, lupus, malignancy
• Subepithelial deposits + IgG + GBM spikes
• Renal vein thrombosis risk (anticoagulate if albumin < 2.0-2.5)
• Treatment: rituximab MENTOR 2019, Ponticelli regimen, CNI

340.2.1.4 IgA Nephropathy

• Globally most common GN
• Synpharyngitic gross hematuria classic
• Mesangial IgA deposits
• MEST-C scoring
• Treatment: ACE/ARB, SGLT2i, Nefecon budesonide (FDA 2021), steroids for severe, sparsentan PROTECT, iptacopan APPLAUSE-IgAN

340.2.1.5 Lupus Nephritis

• ISN/RPS Class I-VI
• Class IV (diffuse proliferative) most aggressive
• Treatment:
  • Induction: MMF or cyclophosphamide + steroids
  • Voclosporin (AURORA 2020) added
  • Belimumab (BLISS-LN 2020) added
  • Hydroxychloroquine all SLE

340.2.1.6 ANCA-Associated Vasculitis

• GPA (PR3, c-ANCA): ENT + lung + kidney
• MPA (MPO, p-ANCA): kidney + lung capillaritis
• EGPA (MPO mostly): asthma + eosinophilia + neuropathy
• Pauci-immune crescentic GN
• Treatment: rituximab + steroids (PEXIVAS) or cyclophosphamide
• Avacopan (C5aR antagonist) FDA 2021 adjunct

340.2.1.7 Anti-GBM (Goodpasture)

• Pulmonary-renal syndrome
• Linear IgG along GBM
• Young men + smokers
• Treatment: plasmapheresis (daily 14-21 d) + cyclophosphamide + steroids
• Rituximab alternative

340.2.1.8 MPGN / C3G

• Immune complex (lupus, hep C, monoclonal) or complement-mediated
• C3G: dense deposit disease (DDD) or C3GN
• Treatment: address underlying; IS; iptacopan (APPLAUSE) emerging

340.2.1.9 DKD

• Globally most common CKD/ESKD cause
• Kimmelstiel-Wilson nodules
• 4 pillars: ACE/ARB + SGLT2i + finerenone + GLP-1

340.2.1.10 AL Amyloid

• Plasma cell dyscrasia (myeloma)
• Light chain restricted
• Daratumumab + CyBorD (ANDROMEDA)

340.2.1.11 Key Trials

• MENTOR (2019): rituximab for MN
• TESTING (2017, 2022): steroids for IgA
• DUPLEX (2023): sparsentan FSGS
• PROTECT (2023): sparsentan IgA
• APPLAUSE-IgAN: iptacopan IgA
• NefigaRD (2021): Nefecon IgA
• BLISS-LN (2020): belimumab lupus
• AURORA (2020): voclosporin lupus
• PEXIVAS (2020): plasmapheresis ANCA
• RAVE, RITUXVAS: rituximab ANCA

340.2.2 易混淆比較

Disease	Immunofluorescence	EM	Treatment
MCD	Negative	Foot process effacement	Steroids
FSGS	IgM, C3 (segmental)	Foot process effacement + sclerosis	Steroids, CNI, rituximab
MN	Granular IgG + C3	Subepithelial dense deposits + spikes	Rituximab, Ponticelli
IgA	Mesangial IgA	Mesangial deposits	ACE/ARB, Nefecon
Lupus	“Full house” (IgG, IgM, IgA, C3, C1q)	Variable	MMF + steroids
ANCA	Pauci-immune (sparse)	Crescents	Rituximab + steroids
Anti-GBM	Linear IgG	Crescents + necrosis	Plasmapheresis + cyc + steroids
MPGN	Granular IgG + C3	Subendothelial deposits	Address underlying + IS
C3G	Dominant C3	Dense deposits or granular	Iptacopan emerging
AL amyloid	Light chain restricted	Fibrils 8-12 nm	Daratumumab + CyBorD

340.2.3 Special Topics

340.2.3.1 APOL1 Risk Variants

• G1, G2 alleles
• African ancestry
• ↑ FSGS, HIV-AN, lupus nephritis, hypertensive nephropathy
• Inaxaplin: targeted therapy (AMPLITUDE trial)

340.2.3.2 Collapsing GN

• FSGS variant
• Worst prognosis
• HIV, COVID-19, parvovirus B19
• APOL1 risk

340.2.3.3 IgA Vasculitis (HSP — Henoch-Schönlein Purpura)

• Children
• IgA deposits in skin, joint, GI, kidney
• Purpura + abdominal pain + arthritis + nephritis
• Treatment: supportive; steroids for severe

340.2.3.4 Cryoglobulinemic Vasculitis

• Hep C most common (Type II + III)
• Skin, joint, neuropathy, kidney (MPGN-like)
• Treatment: DAA for hep C; rituximab for severe

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