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32 Cards in this Set
- Front
- Back
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Renal System- Glomerulonephritis by Leonard
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Renal System- Glomerulonephritis by Leonard
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Nephritic
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-itic=inflammatory (like -itis)
manifestations are Hematuria, azotemia, variable proteinuria, oliguria, edema, and hypertension |
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Ok, fine, so what's NEPHROTIC?
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>3.5 gram/day proteinuria, hypoalbuminemia, hyperlipidemia, lipiduria
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Minimal Change Disease (MCD)
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-Most common form of nephrotic syndrome in children (~90%)
- Tends to be fairly “albumin selective” proteinuria (albumin >>Ig) Morphology LM: normal glomerulus IF: negative EM: effacement of podocyte foot processes (therefore disrupted filtration slits); no immune deposits good prognosis, use corticosteroids for tx |
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Membranous Glomerulopathy
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-Most common cause of nephrotic syndrome in adults (Caucasians, Asians)
Morphology: LM: thickened capillary walls IF: confluent granular staining for IgG EM: electron dense sub-epithelial immune deposits Prognosis ~25% progress to ESRD (won't return function) |
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Membranous GN... more cells, right?
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Thicker. Not proliferative process. Increased deposition of glomerular basement membrane material and ECM in response to injury of immune complex deposition
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Focal Segmental Glomerulosclerosis (FSGS)
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-Scarring of portion of some glomeruli
Primary: at least 4 different variants based on morphology Secondary: same variants, but due to extrarenal disease process -Obesity, sickle cell disease, cyanotic heart disease, HIV, IV drug abuse -E.g., collapsing variant (seen in HIV-associated nephropathy): very aggressive (ESRD in < 1 yr) *decrease oxygen exacerbates the injury problem |
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Clinical features of FSGS
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Gradual (insidious), Causes ~30% of nephrotic syndrome in adults (most common cause in African-Americans)
morphologic:May be confused with MCD on biopsy as pathologic features are focal and segmental EM: diffuse effacement of foot processes, loss of podocytes and collapse of capillaries with increased ECM |
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Diabetic Glomerulosclerosis (GS)
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Associated with small vessel disease (sclerosis) throughout body.
clinical- presents as mucroalbuminuria; seen in 50% of all diabetic pts. etiology- generalized increase in BM material synthesis within microvasculature. *secondary disease |
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when you see Kimmelstiel-Wilson nodules, you think of...
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diabetic GS.
-(PAS- and silver- positive acellular, nodular mesangial sclerosis) -Hyaline arteriololar sclerosis Prognosis ~ 30% develop ESRD (leading cause in USA) |
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Amyloid Nephropathy
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rare:
Clinical: Renal involvement typical in either AA or AL forms of systemic amyloidosis AA amyloid: associated with chronic inflammatory process AL amyloid: associated with plasma cell/B cell neoplasm, derived from light chains of Ig molecules (multiple myeloma) morphology: LM: congo red stain tx:AA amyloidosis – potent anti-inflammatory agents (e.g., colchicine) Tx: AL amyloidosis – chemotherapy used to treat multiple myeloma |
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5 types of Nephritic Syndrome
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1. Acute post-infectious glomerulonephritis
2. Membranoproliferative glomerulonephritis -Type 1 and Type 2 3. Lupus nephritis 4. IgA nephropathy 5. Anti-GBM glomerulonephritis -With lung involvement = Good pasture disease |
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One of most common pediatric kidney diseases (nephritic)
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Acute Post-Infectious GN (aka acute proliferative GN; acute post-streptococcal GN)
Etiology: -Most often related to nephritogenic strains of group A strep -Type III hypersensitivity rxn (immune complexes); 2-4 weeks post pharyngitis or skin infection |
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What do you see on the light miscoscope during acute post-infectious GN? EM? Prognosis?
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Morphology:
LM: proliferative GN Early: increase mesangial matrix with increase PMNs Late: mildly hypercellular glomeruli IF: “lumpy-bumpy” IgG, C3 around capillaries, mesangium EM: sub-epithelial humps Prognosis Majority improve to baseline within months |
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Membranoproliferative GN (MPGN)
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-Proliferation of glomerular cells, alteration in GBM, infiltration by WBCs
Primary MPGN is divided into two groups -Type I MPGN -Type II MPGN (a.k.a., Dense Deposit Disease) Secondary (systemic): SBE; osteomyelitis; HCV; HBV; neoplasia; a1-antitrypsin deficiency |
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Type I MPGN: who gets it?
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Clinical:Can occur at any age; most frequent in older children and younger adults
-More prevalent in underdeveloped countries with high prevalence of chronic infections -Often have low levels of complement (C3) Etiology: Deposition of immune complexes in mesangium and subendothelial capillary walls -Mesangial hypercellularity and remodeling -Typically there is chronic antigenemia |
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Type I MPGN : morphology
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-Glomerular crescents (bad sign)
-Silver stain: doubling (“replication”) of GBM = “tram-tracking” Prognosis Treatment of underlying disease process (if secondary) ~ 50% patients progress to ESRD |
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Type II MPGN: Dense Deposit Disease... etiology.
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Clinical: Rare; similar to Type I MPGN, except:
More pronounced hypocomplementemia Prognosis slightly worse Etiology: Extensive deposition of complement in GBM and mesangial matrix; virtually no Ig (NOT immune complex) -Mutations in alternative complement regulatory factors (e.g., factor H) -Most patients have IgG autoantibody, the C3 nephritic factor (stabilizes activated C3 convertase (C3bBb) |
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Type II MPGN: Dense Deposit Disease... morphology and treatment
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Morphology:
LM: similar to type I MPGN, but often with less pronounced hypercellularity IF and EM: “ribbon-like” zone of increased density within the GBM and mesangial matrix Prognosis: Lack of effective treatment with variable progression Some patients develop numerous glomerular crescents and picture of rapidly progressive GN (RPGN) ~ 50% develop CRF within 10 yrs Relatively high recurrence within transplanted kidneys |
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Lupus Nephritis
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-SLE is autoimmune disease with generally cross-reacting or many different antibodies
-Nephritis is one of more common features of SLE (70%) -Treatment is with immunosuppression (corticosteroids, etc.) *wire loop appearance |
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What's the most common form of glomerulonephropathy in the WORLD?!
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IgA Nephropathy (Berger Disease)
Symptoms vary: 40% asymptomatic microscopic hematuria 40% intermittent gross hematuria 10% nephrotic syndrome 10% renal failure -Generally slowly progressive course |
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IgA Nephropathy etiology...
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Deposition of IgA-dominant immune complexes
-Patients often have high levels of circulating IgA -Symptoms initiated or exacerbation with respiratory or GI infections Infections may stimulate IgA production in respiratory tract or gut; these Ab’s could be nephritogenic |
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Anti-GBM GN + pulmonary hemorrhage=
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Goodpasture Disease
rare, but aggressive |
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Anti-GBM GN, etiology, moprhology
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Etiology:
-Often follows URI -Autoantibody directed against portion of type IV collagen molecule present in GBM --Antigenic epitope also may be present in pulmonary alveolar capillary BM -----Pulmonary involvement seems to be present in context of some kind of injurious factors (e.g., cigarette smoking) Morphology: LM: glomerular crescents (>90%) – feature of RPGN IF: diffuse linear staining of GBM for IgG |
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Rapidly Progressive GN (RPGN)
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A.K.A. Crescentic GN
Severe glomerular injury with rapid and progressive renal dysfunction, severe oliguria, signs of nephritic syndrome, and ultimate fatality if not treated |
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Three Types of RPGN
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TYPE I (ANTI-GBM ANTIBODY)
-Goodpasture Syndrome TYPE II (IMMUNE COMPLEX) -Lupus nephritis -Henoch-Schönlein purpura (IgA nephropathy) TYPE III (PAUCI-IMMUNE) |
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RPGN Morphology and Prognosis. What do you see in the light microscope?
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Morphology:
LM: presence of crescents in most of the glomeruli Proliferation of parietal epithelial cells, with compression and eventual obliteration of the glomerular capillary tuft Prognosis: Generally not good, somewhat dependent upon underlying cause |
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Hereditary Nephridites, what does it generally present with? What are the two types?
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Generally present with hematuria
1. Thin basement membrane disease 2.Alport syndrome |
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Most common cause of hereditary hematuria
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Thin basement membrane disease.
Clinical: Asymptomatic hematuria Etiology: mutations in genes coding for alpha chains of type IV collagen Morphology: EM shows uniform thinning of GBM Prognosis: generally benign |
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Hematuria with progression to CRF is...
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Alport syndrome
Also: nerve deafness; eye disorders (lens ectopia, cataracts) Etiology: Majority are X-linked (males express full form; females limited to benign hematuria) --Defective assembly of collagen type IV (GBM, BM of eye lens, cochlea) Morphology: EM: GBM thinning early, later there is alternating thinning and thickening of GBM with “fracturing” of GBM |
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Chronic Glomerulosclerosis (CGN). Clinical features. Etiologies?
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Conglomeration of end-stage forms (ESRD) of various glomerular diseases
Clinical: Progressive renal failure -Oliguria, proteinuria, edema, azotemia, uremia, death -Anemia (deficient EPO), anorexia, general malaise -Most patients are hypertensive with cardiac and CNS effects Etiologies: Vary, depending upon the underlying cause -Those who survive acute phase of RPGN generally develop CGN and CRF |
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CGN Morphology
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Shrunken kidney with diffuse granular cortical surface
-Cortex is thinned |
