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9 Cards in this Set
- Front
- Back
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Minimal Change Nephropathy
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-accounts for Nephrotic syndrome:
90% children; 20% adult -associated with atopy (eczema, asthma, hayfever) -often follows URT infection -minimal change because: -normal light microscopy & immunostaining -EM shows fused podocyte foot processes -responds well to steroid -if relapse, ciclosporins -Renal impairment doesn't occur -NSAIDs can cause minimal change disease |
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Focal Segmental Glomerulosclerosis
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-accounts for 15% of adult nephrotic syndrome
-can cause hematuria & hypertension -focal & segmental scarring, containing complement & Ig -obliterated capillary lumen -hyaline & lipid deposits -EM shows fused podocyte foot processes -poor response to steroid, relapse may be reduced by ciclosporin or cyclophosphamide -50% patients -> ESRF within 10yrs of dx -disease can recur with renal transplant -recognised cause = Obesity |
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Membranous GN/nephropathy
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-accounts most of adult nephrotic syndrome
-proteinuria & often renal impairment -Histo: diffusely thickened GBM & subepith Ig deposits along GBM -Effacement of Podocyte foot processes IF: GRANULAR deposit of Ig & complement -usually idiopathic -often secondary to malignancy of lung & colon, hepB, SLE, or use of gold or penicillamine drugs -some respond to steroid, cyclophosphamide -minority develops ESRD |
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Membranoproliferative GN
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-uncommon
-mainly occurs in young adult & children -varied presentation: aysmptomatic hematuria or proteinuria, or usual presentation with combined nephrotic & nephritic syndromes -most progress to ESRF -no useful tx -there are 2 types Light microscopy [both types similar]: -Incr'd mesangial cell & mesangial matrix; endoth cells, and leukocytes -Thickened GBM -accentuated lobular structure -mesangial interposition gives the appearance of split GBM Differ in IF & ultrastructural microscopic features -Most: type 1 (80%) - "subendoth" & mesangial immune deposits -IF: C3 in irreg GRANULAR pattern -assoc'd with hepB, infection, SLE -low C3&4 due complement activation & depletion -present: IgG and early complement components -Rare: type 2 (20%) - "intramemb" immune deposits -IF: C3 irreg chunky & segmental linear foci -due antibody that activate & deplete complements -absent: IgG and early complement components |
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IgA Nephropathy
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-most common primary glomerular disease
-children & young adults -frank hematuria 1-2d after non-specific URT infection -can also present with asymptomatic microscopic hematuria, proteinuria and renal impairment -Histo: incr'd mesangial cells & mesangial matrix -IF: GRANULAR IgA deposit in "mesangium" -often have raised serum IgA -abN production & clearance of IgA -incr'd synth in response to respir/GI exposure to environmental agents ->igA & IgA-containing immune complexes in the mesangium -> activate alternative complement pathway ->glomerular inj -unsuccessful tx -30% develop ESRF -recur with renal transplant |
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Crescentic GN
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-Clinical syndrome and not a specific etiologic form of GN
-crescents = indicate severe glomerular dmg; due proliferation of parietal epithelial cells of Bowman’s capsule in response to injury and by infiltration of monocytes and macrophages, and fibrin [believed to be stimulus] enters thru breaks in the GBM -IF: depends which type -main causes: -anti-GBM disease (Goodpasture's syndrome) -systemic vasculitis -SLE -IgA nephropathy -vasculitis -untreated, death from renal failure in wks-mo |
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Crescentic GN - In most cases glomerular injury is immunologically mediated, and where not idiopathic, it is classified into:
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25% = Type I: Anti-GBM Antibody CrGN
-associated with anti-GBM antibodies with a LINEAR immunofluorescence (IF) deposition pattern 25% = Type II: Immune complex-mediated CrGN -have a lumpy, GRANULAR IF deposition of antibodies typical of post-streptococcal infection. 50% = Type III: Pauci Immune CrGN -show no antibody deposition. Most of these patients show anti-neutrophil cytoplasmic antibodies (ANCA) in their serum which plays a role in some of the vasculitides. |
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Post-infectious GN
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-typically 1-4wks after grpA beta-hemolytic strep infection; high serum ASO titre
-antigen/antibody complex initiate inflam by complement and leucocyte pathways -proliferation: endothelial & mesangial cells; neutrophils, monocytes -v few capillary lumena visible -"subepith" electron dense deposit -IF: GRANULAR pattern of IgG and complement within capillary wall & some mesangial area; -small no of children ->rapidly progressive GN -adults: 15-50% ESRD over next 10/20 yrs [chronicity low in children] |
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Anti-GBM disease (Goodpasture's syndrome)
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-best eg of "Nephritis due in situ immune complex"
-rare <1% human GN cases -antibodies are directed against fixed antigens in the GBM -due autoimmune antibodies against type4 collagen in GBM -and also the alveolar BM in lung [=Goodpasture syndrome] -binding of antibody to these membranes cause inflammation -this causes rapidly progressive crescenteric GN with ARF & lung hemorrhage -usually male with HLA-DR15 -untreated die from pulm hemorrhage or renal failure -IF: LINEAR -tx: -plasma exchange (remove antibodies) -immunosuppression with steroids & cyclophosphamide (inhibit glomerular inflam & reduce antibod production) -early tx most recover and relapse uncommon |
