339.4 ð ç« æ«éèš Summary
339.4.1 ð äžå¥è©±çžœçµ
Glomerular diseases = åœ±é¿ glomerulus ççŸç ïŒnephrotic syndrome ç¶å ž 5 倧ç¹åŸµ = proteinuria > 3.5 g/24h + hypoalbuminemia + edema + hyperlipidemia ± hypercoagulabilityïŒnephritic syndrome = hematuria + RBC casts + HTN + AKI variable + edema + proteinuria variableïŒtop nephrotic causesïŒMCD (children most), FSGS (adults in US most common), MN (older adults â anti-PLA2R 70-80%), DKD (globally most common), amyloidosis, MPGNïŒsecondary causes hep B/C, HIV, drugs (NSAIDs, lithium, ICI), cancer (paraneoplastic), pregnancyïŒtop nephritic causesïŒIgA nephropathy (globally most common), PSGN, lupus nephritis (Class I-VI ISN/RPS), ANCA-associated (GPA, MPA, EGPA), anti-GBM (Goodpasture), MPGN/C3G, IgA vasculitis (HSP), cryoglobulinemic (Hep C)ïŒRPGN 3 types: anti-GBM (Type I) + immune complex (Type II â lupus, post-infectious) + pauci-immune (Type III â ANCA)ïŒworkupïŒUA + UACR + 24h urine + serum chemistry + immunology (ANA, dsDNA, ANCA, anti-GBM, anti-PLA2R, ASO, complement C3/C4, RF, cryoglobulin) + hepatitis + HIV + free light chains â renal biopsy gold standard (LM + IF + EM)ïŒsupportive treatmentïŒACE/ARB (reduce proteinuria + BP), diuretics, statins, anticoagulation for high-risk nephrotic (MN with albumin < 2.0-2.5), vaccinations (pneumococcal); specific therapy tailored to GN (Ch339)ã
339.4.2 ð æ²»ç粟èŠ
- supportive (all GN)ïŒACE/ARB (reduce proteinuria + BP) + low-Na diet + diuretics (loop ± thiazide) + statins + anticoagulation if high-risk + vaccinations + avoid nephrotoxins
- nephrotic anticoagulationïŒMN with albumin < 2.0-2.5 g/dL â LMWH or DOAC; continue until proteinuria resolves (severe hypoalbuminemia â thrombosis)
- immunosuppression (specific to GN) in Ch339ïŒsteroids, cyclophosphamide, rituximab, MMF, CNIs (tacrolimus, cyclosporine), eculizumab/ravulizumab (aHUS), belimumab (lupus), voclosporin (lupus), sparsentan (FSGS/IgA)
- vaccinations critical (especially nephrotic â encapsulated organism risk)ïŒPCV20 + PPSV23, annual flu, COVID, RSV ⥠60, hepatitis B
339.4.3 ð¯ ç§é«åž«çèåæé
- nephrotic syndrome 5 倧ç¹åŸµïŒproteinuria > 3.5 g/24hãhypoalbuminemia < 3.5 g/dLãedemaãhyperlipidemia (â LDL + TG)ãhypercoagulability (esp renal vein thrombosis from antithrombin III loss)
- nephritic syndrome æšèªïŒhematuria + RBC casts + dysmorphic RBC + HTN + AKI variable + edema + proteinuria variable (often sub-nephrotic)
- top nephrotic causesïŒMCD (children 80%), FSGS (adults US), MN (older â anti-PLA2R 70-80% diagnostic), DKD (globally most), amyloid
- top nephritic causesïŒIgA nephropathy (globally most), PSGN (kids), lupus nephritis (Class I-VI), ANCA (GPA/MPA/EGPA), anti-GBM (Goodpasture), MPGN/C3G
- RPGN 3 types (Couser)ïŒType I anti-GBMãType II immune complex (lupus, post-infectious, IgA)ãType III pauci-immune (ANCA); éœæ crescents on biopsy
- renal biopsy is gold standardïŒlight microscopy + immunofluorescence (pattern key) + electron microscopy (deposits + foot process effacement)
- anti-PLA2R primary MN 70-80%ïŒuseful for diagnosis + monitoring + treatment response correlates with antibody decline
- C3 low diseases: PSGN, lupus nephritis, MPGN/C3G, cryoglobulinemia (Hep C) â important differential
- nephrotic anticoagulation indicationsïŒMN with albumin < 2.0-2.5 g/dL â LMWH or DOAC; high renal vein thrombosis + DVT/PE risk
- vaccinations critical in nephrotic syndromeïŒencapsulated organism risk (S. pneumoniae) â PCV20 + PPSV23ïŒflu + COVID + RSV + hep B