Pathology of Nephritic Syndrome Flashcards by A B

Asymptomatic proteinuria/hematuria

1) symptoms
2) Urinalysis shows…

1) asymptomatic
2) proteinuria (> 150 mg/24 hr)

hematuria = dark or normal color urine + RBCs + red cell casts

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Etiologies of asymptomatic proteinuria/hematuria

Proteinuria = leakage of protein thru glomerular basement membrane (glomerular disease) NOT TUBULAR

Hematuria = red cell casts = glomerular bleeding

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Causes of leakage in basement membrane and what leaks thru

1) loss of electrostatic neg charge barrier so albumin leak thru
2) loss of size barrier so albumin, globulin and protein leak thru

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Acute nephritic syndrome

1) signs and due to?
2) how does GFR change
3) effects of change in GFR
4) time frame

1) hematuria and proteinuria due to incr glomerular capillary permeability
2) decr GFR
3) Na+/H2O retention –> edema, CHF, HTN

azotemia (incr creatinine and BUN)

hyperkalemia

4) hours to days

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Rapidly progressive nephritic syndrome

1) signs and due to?
2) how does GFR change
3) effects of change in GFR
4) time frame

1) hematuria and proteinuria due to incr glomerular capillary permeability
2) VERY DECR GFR
3) more fluid retention, more azotemia, oliguria
4) longer version of acute nephritic

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Nephrotic syndrome

1) signs and due to?
2) effect on plasma oncotic pressure
3) effects of change in oncotic

1) massive proteinuria (> 3.5 g/24 hrs) not compensated by hepatic albumin syntheiss
2) decr plasma oncotic pressure
3) Na+ and H2O retention, edema, hypercholesterolemia

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Chronic renal failure

1) due to
2) how does it affect GFR
3) assoc with
4) etiologies

1) nephron loss
2) decr GFR
3) uremia
4) glomerular disease, vascular disease/HTN (MOST COMMON), infection, drugs/toxins, UTI

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Morphologic pattern of glomerular disease

1) mesangial, endocapillary, epithelial (podocyte) = crescent proliferation
2) leukocyte infiltration = PMN’s or leukocytes
3) basement membrane thickening/changes
4) sclerosis (segmental or global)

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indicative of…

glomerular disease

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Distinguish between focal/diffuse sclerosis

Of all total glomeruli

focal = some normal, some abnormal

diffuse = affects all glomeruli

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Distinguish between segmental/global sclerosis

within individual glomerulus

segmental = only portion of one

global = entire glomerulus

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Things to identify on immunofluorescence

1) Ig’s/complement
2) distribution (mesangial/capillary)
3) pattern (linear/granular)

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Things to identify on electron microscopy

1) deposits of Ag-Ab complexes
2) GBM changes

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Things to identify on serum

1) complement levels (decr in some acute GN’s)
2) ANA- SLE
3) ASO (anti-strep O) = post-strep GN
4) ANCA = Wegener’s, PAN

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IF you see ASO on serum what could that indicate?

If you see ANCA on serum what could that indicate?

1) post-strep GN
2) Wegener’s, PAN

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Thin Basement Membrane Disease

1) also known as
2) prognosis
3) mutations in…
4) diagnsois based on …

1) benign familial hematuria
2) excellent
3) alpha chains of collagen IV (thin basement membrane)
4) electron microscopy)

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Alport’s disease

1) triad of …
2) due to mutation in…
3) inheritance
4) can’t form …
5) definitive diagnosis based on what?
6) progress to…

1) nephritis, deafness, ocular lesions
2) alpha-5 chain of collagen IV
3) X-linked
4) normal basement membrane
5) basket-weave pattern on EM (abnormal lamina densa)
6) ESRD

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Signs of non-inflamm pathogenesis DUE TO OXIDATIVE

1) mediated by…
2) immune complexes activ …
3) damages …

1) circulating factors or Ig’s that bind to membranes of glomerular epithelial cells
2) activ GEC to release reactive oxidants/proteases that damage diaphragms btwn podocytes
3) cause GEC to detach from basement membrane –> leak

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Signs of non-inflamm pathogenesis DUE TO COMPLEMENT

1) mediated by…
2) antibodies bind…
3) damages …

1) complement-fixing anti-GEC antibodies
2) antibody bind GEC –> activ complement membrane attack complex
3) poke hole in glomerular basement membrane, incr permeability

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Inflammatory pathogenesis of glomerular disease usually involves (4)

1) PMN’s
2) macrophages
3) mesangial cells
4) T cells

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how are PMn’s involved in leading to inflamm pathogenesis

1) Ig antibody deposits within capillaries
2) activ complement
3) infiltrate of PMN’s
4) PMN’s burst –> release ROS –> damage glomerular basement membrane and endo cells
5) fibrin deposit in Bowman’s space –> form CRESCENTS

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if crescents form what does that indicate

fibrin in bowman’s space –> severe inflammation

If Ig bind to endogenous antigens, what diseases

If Ig bind to EXOGENOUS antigen, what disease

1) SLE, good pasture’s
2) infection or toxin

macrophage role in inflamm disease

1) macrophage activ by cytokine
2) release oxidants and proteases that damage glomerular basement membrane
3) macrophage release cytokines that stim sclerosis

mesangial cell role in inflamm disease

1) immune complex, complement, cytokine activ mesangial cell 2) release oxidants, proteases, growth factors, ECM

T cell role in inflamm disease

1) T cells activate macrophage and mesangial cells

What is endocapillary hypercellualrity mean? What is the marker of mesangial hypercellularity what are crescents a reaction to?

both mesangial and endothelial cells in vascular compartment \> 2-3 mesangial nuclei per island severe injury to glomerular capillaries --\> fibrosis in Bowman's space

Describe cellularity, segmental vs.global, capillary loops

Hypercellular? yes, mesangial hypercellularity Segmental or global? segmental Capillary loops still open

Left = immune complexes deposited in mesangial region podocytes intact basement membrane intact Right = IgA deposited in mesangial regions

IgA neuropathy 1) usually presents as ... 2) often coincides with ... 3) morphology on stain 4) on EM, on IF 5) therapy 6) prognosis

1) asymptoamtic hematuria 2) nephritic or nephrotic; with URI or GI infection, or liver disease 3) mesangial, focal (can be MPGN) 4) EM = mesangial deposits IF = mesangial IgA 5) ? steroids 6) ESRD

Henoch-Schonlein Purpura 1) also known as 2) due to ... 3) may involve 4) renal biopsies look like ... 5) patient population

1) systemic IgA vasculitis 2) systemic deposition of IgA immune complex 3) kidney, skin, joint, GI 4) IgA nephropathy but more endocapillary prolif or crescents 5) \< 10 yrs post URI esp strep

Left = hypercellular, no open capillary, inflamm cells (PMN's) creatinine = 2.5, RBC and RBC casts in urine = nephritic Right = diffuse global endocapillary proliferative GN

Subendothelial Hump-like depositis left: red = humps right = fenestrated endothelium too many cells inside

left = hypercellularity in capillary loop subepithelial deposits right = starry sky pattern = granular with IgG/C3 immune complexes

Postinfectious GN 1) usually due to 2) acute nephritic or nephrotic 3) morphology 4) on EM, IF, serology 5) therapy 6) \_\_% of children recover, __ % OF ADULTS recover

1) post strep infection 2) acute nephritic 3) diffuse prolif, exudative GN 4) EM = subepithelial hump-like deposits IF = granular GBM = starry sky serology = elev ASO 5) supportive 6) 99, 60

left = necrotizing and crescent glomerulonephritis karyolectic debris right = proliferation = crescents in bowman's space

left = ruptured capillary loop

Crescentic glomerulonephritis 1) crescents = sign of \_\_\_ 2) caused by \_\_\_ 3) present as \_\_\_ 4) more crescents correl with \_\_\_ 5) glomeruli will usually \_\_\_

1) severe acute glomerular disease 2) fibrinoid necrosis of capillaries 3) RPGN 4) serum creatinine levels and prognosis 5) heal with scar

FSGN and Crescentic glomerulonephritis If you see linear staining on IF ... IF you see granular staining ... If you see no staining ...

1) goodpasture's, anti-GBM 2) IgA, SLE, endocarditis, Idiopathic 3) wegener's, microscopic PAN, churg-strauss, idioapthic

left = linear staining for IgG right = starry sky = postinfectious GN presents as rapidly prolif

FSGN, crescentic GN Serum/blood studies 1) if you see anti-GBM antbodies... 2) ANA, anti-dsDNA, complement depletion (C3, C4) 3) ANCA (MPO- cationic protein fixing complement) 4) blood cultures

1) goodpasture 2) lupus 3) vasculitis 4) endocarditis

Anti-gbm disease 1) ___ IgG antibody 2) antibodies directed against \_\_\_ 3) if renal limited, \_\_\_ 4) if renal + lung, \_\_\_ 5) classic presentation = 6) on IF?

1) nephrotoxic 2) alpha chain of collagen IV in basement membrane 3) anti-GBM 4) goodpasture 5) RPGN + hemoptysis 6) linear

Crescentic GN 1) usually \_\_ 2) therapy 3) prognosis

1) RPGN 2) steroids, cytotoxics, plasmaphoresis 3) poor

lupus nephritis 1) part of \_\_\_ 2) IF shows... 3) present as ... 4) prognosis

1) SLE 2) full house = granular with positive IgG, IgA, IgM, C1q, C3 3) nephritic syndrome, RPGN, nephrotic syndorme, or mixed nephritic-nephrotic 4) usually good with treatment

anti-gbm disease

left = diffuse glomerulonephritis, hypercelllular, endocapillary prolif, PMN's thick peripheral capillary loop right = wire loop lesions= very red and huge deposits

Normal glomerulus is 70% \_\_\_ Largely contains \_\_\_ Features of mesangial cells

1) space 2) largely mesangial cells 3) derived from monocytes; produce matrix that surrounds self and interdigitates with capillary loops; phagocytose; intravascular

what is the most selective barrier for filtration made of? what is the largest filterer of proteins

slit diaphragm = podocytes connected by thin line slits made of nephrin (2 nephrins holding hands + adhesion molec holding podocytes to basement membrane basement membrane = leaky so podocytes are most stringent

nephrotic syndrome 1) presents with \_\_\_ 2) due to \_\_\_ 3) leads to...

1) massive proteinuria (\> 3.5 g/24 hr) 2) decr plasma oncotic pressure 3) severe edema, hypercholesterolemia, lipiduria

Hyperfiltration causing glomerular disease seen in ..

diabetics segmental glomerulosclerosis

loss of glomerular polyanions how cause glomerular disease

incr permeability of glomerular capillary wall --\> lose albumin in urine --\> decr plasma oncotic pressure --\> fluid into extravascular