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CPR II Exam 3 > Chapter 20: Renal Pathology Part 1 > Flashcards

Chapter 20: Renal Pathology Part 1 Flashcards

1
Q

What is the difference between Azotemia (Prerenal vs Postrenal) and Uremia?

A

Azotemia: elevated BUN and Creatinine lvls

  • Prerenal: from hypoperfusion (BUN/Cr > 20)
  • Postrenal: from obstruction distally (BUN/Cr < 20)

Uremia: azotemia associated with clinical signs and symptoms (metabolic/endocrine dysfunctions)
- failure of renal excretory function

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2
Q

Nephritic vs Nephrotic Syndromes

How do they typically present?

A

Nephritic - more HEMATURIA (sicker pts)

  • presents with hematuria and hypertension
  • seen in acute poststreptococcal glomerulonephritis

Nephrotic - more PROTEINURIA (> 3.5 g/24 hrs)

  • bad proteinuria, hypoalbuminemia, hyperlipidemia
  • inc. edema (periorbital), dec. HDL
  • inc. risk of infection and hypercoagulable state
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3
Q

What is the difference between Acute Kidney Injury, Chronic Kidney Injury, and End-Stage Renal Disease?

A

AKI: rapid GFR dec. (hrs to days) with fluid/electrolyte disreg. and retention of metabolic wastes
- MCC by ACUTE TUBULAR INJURY

CKI: diminished GFR persistently < 60 ml/min for at least 3 months
- MCC by diabetes and hypertension

ESRD: GFR < 5% of normal (terminal stage of uremia)

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4
Q

What is the difference between UTIs affecting the kidney vs bladder?

A

Kidney: PYELONEPHRITIS
- fever IS present

Bladder: CYSTITIS
- fever is NOT present

urinary tract infections are characterized by bacteriuria and pyuria (bacteria and leukocytes in urine

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5
Q

What is the difference between Acute Glomerular Response to injury vs the Chronic Glomerular Response to injury?

A

Acute: HYPERCELLULARITY

  • inc. # of cells in glomerular tuft
  • formation of CRESCENTS if severe enough

Chronic: BM thickening, hyalinosis, sclerosis

  • BM: PAS stain shows thickening
  • hyalinosis = END STAGE of damage (eosinophilic)
  • sclerosis: Trichrome Blue stain (ECM deposits)
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6
Q

Pathogenesis of Glomerular Injury

What is the difference between a Granular pattern on immunofluorescence vs a Linear pattern on immunofluorescence and what is a common example of each?

A

Granular = deposition of immune complexes
- Ex: membranous nephropathy

Linear = auto-abs against components of the GBM
- Ex: Goodpasture syndrome

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7
Q

What is the difference between these patterns of glomerular damage:

  1. Diffuse
  2. Focal
  3. Segmental
  4. Global
A
  1. involves ALL of the glomeruli
  2. involves a SUBSET of the glomeruli
  3. only certain PORTIONS of affected glomeruli are involved
  4. ENTIRETY of affected glomeruli is involved
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8
Q

What are the two major Nephritic Primary Glomerulonephritides and what are the 3 major Nephrotic Primary Glomerulonephritides?

A

Nephritic

  • Acute Proliferative Glomerulonephritis
  • Rapidly Progressive Glomerulonephritis

Nephrotic

  • Membranous Glomerulopathy
  • Minimal change Disease
  • Focal Segmental Glomerulsclerosis (FSGS)

FSGS can be MIXED - both nephrotic and nephritic

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9
Q

What is the most common cause of nephrotic syndrome in adults?

What has a greater link to decline of renal function than severity of glomerular injury and how does it develop?

A

FSGS = Focal Segmental Glomerulosclerosis

when a pt presents with Nephrotic Syndrome but also has Nephritic Syndrome = FSGS

  • TUBULOINTERSTIAL FIBROSIS has stronger correlation to severity of glomerular injury
    • either tubule infarct or direct injury to tubule cells
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10
Q

Acute Proliferative Glomerulonephritis

What is it, how does it develop (what 3 strains), and what does it look like morphologically?

A
  • diffuse proliferation of glomerular cells associated with influx of leukocytes; usually immune complexes
  • from Post-Strep A (pyrogenes) or Skin infection 1-4 WEEKS AFTER untreated case due to Strep A strains 12, 4, 1; Ab formation against pyrogenic exotoxin B (SpeB) –> Ags are planted in capillary walls

M: enlarged, hypercellular glomeruli with “hump-like” GRANULAR deposits in subEPITHELIAL space; contains IgG/IgM/C3; tubules w/RBC casts

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11
Q

Acute Proliferative Glomerulonephritis

Who does it present in typically, how does it present, and what titers are elevated?

A
  • usually seen in CHILDREN (6-10 ys) with sudden malaise, fever, nausea, periorbital edema, tea-colored urine 1-2 WEEKS AFTER Strep A infection
    • worse prognosis in ADULTS
  • labs show elevated ASO titers and low serum complement lvls (consumption)
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12
Q

Acute Proliferative Glomerulonephritis

How does glomerulonephritis caused by STAPHylococcal infection differ from that caused by STREP infection?

A
  • Staphylococcal infections differ by sometimes producing IgA immune deposits rather than IgG immune deposits
  • will STILL HAVE granular deposits with subEPITHELIAL humps
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13
Q

Rapidly Progressive Glomerulonephritis

What is it, what formations is it associated with, and what are the 3 different types of this disease?

What HLA is Goodpasture Syndrome associated with?

A
  • severe glomerular injury associated with CRESCENT formation in most glomeruli (parietal epi proliferation lining bowman capsule with monocytes/MOs)
    • VERY SICK PATIENTS

Type 1: anti-GBM antibodies (Goodpasture Syndrome)

  • LINEAR Ab deposits to Type IV collagen
  • use plasmapheresis; associated with HLA-DRB1

Type 2: immune complexes (SLE, IgA nephropathy, etc)

  • GRANULAR deposits
  • NO plasmapheresis; treat underlying cause

Type 3: Pauci-Immune; associated with ANCA Abs

  • ANCAs are DIAGNOSTIC (NO IF pattern)
  • Wegener Granulomatosis/microscopic polyangiitis
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14
Q

Rapidly Progressive Glomerulonephritis

What does it look like morphologically (kidneys) and histologically?

A

M: enlarged and PALE kidneys with cortical PETECHIAL HEMORRHAGE

H: CRESCENTS (in Bowman’s Capsule); obliterates URINARY SPACE and compresses the glomerular tuft

Type 1 = linear, Type 2 = granular, Type 3 = NONE

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15
Q

Nephrotic Syndrome

Why do pts. exhibit hyperlipidemia/hypercholesterolemia and what infections are pts at increased risk of developing?

What thrombotic issues do these pts. experience and why?

A
  • liver is trying to compensate for protein loss, so it increases protein synthesis; side-effect is increase in lipoproteins and cholesterol
  • pts usually have loss of endogenous anticoagulants, so renal vein thrombosis is common and can lead to a VARICOCELE on the LEFT in MALE pts. (enlarged scrotum)
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16
Q

Membranous Nephropathy

What is it, how does it develop (HLA and Ag), and what does it look like morphologically?

A
  • diffuse glomerular capillary wall thickening due to chronic (IgG4) immune complex deposition that activates complement (MAC)
    • Primary: idiopathic (HLA-DQA1/Abs to PLA2R) - 75%
    • Secondary: SLE, drugs, tumors, infections
  • MAC complex causes capillary wall injury and protein leakage

M: diffuse uniform BM thickening with dense subEPITHELIAL “spike and dome” deposits with a GRANULAR pattern on immunofluorescence

17
Q

Membranous Nephropathy

How does it present clinically, what does it not respond well to, and what are good biomarkers for screening?

A

C: insidious nephrotic syndrome onset = hematuria/HTN
- proteinuria is NONSELECTIVE (high molecular weight)

  • does not respond well to CORTICOSTEROIDS
  • circulating Abs to PLA2 may be useful biomarkers of disease activity
18
Q

Minimal Change Disease

What is it, what is it the most common cause of, and what disorders is it associated with?

What can usually be seen in the proximal tubules of these patients?

A
  • CHILDHOOD nephrotic syndrome characterized by foot process effacement = MASSIVE proteinuria with NORMAL glomerulus on light microscopy
    • *proteinuria is HIGHLY selective**
    • proximal tubules likely to exhibit LIPID DEPOSITION
  • most common cause of nephrotic syndrome in children (peak 2-6 yrs) and IS responsive to CORTICOSTEROIDS
  • development is unknown, but is associated with other ATOPIC disorders (ECZEMA/RHINITIS)
19
Q

Minimal Change Disease

What two pts. populations are at inc. risk of development, what does this disease look like morphologically, and how does it present clinically?

How can this disease be treated?

A
  • inc. incidence in Hodgkin Lymphoma and in pts. with defects in T Cell-mediated immunity (Reed-Sternberg cells)

M: normal glomerulus on light microscopy, but Electron Microscopy shows lesion in VISCERAL EPITHELIAL cells (diffuse effacement of podocytes)
- IF shows NOTHING (no immune deposits)

C: preservation of renal function w/o hematuria/HTN; SELECTIVE proteinuria (albumin) and is effectively treated with corticosteroids

20
Q

Focal Segmental Glomerulosclerosis

What is it, how does it differ from Minimal Change Disease, and what is its pathogenesis?

A
  • sclerosis of some glomeruli affecting a part of each affected glomeruli with NEPHROTIC syndrome
    • MCC of nephrotic syndrome in ADULTS (AA/Hisp)
  • has NONSELECTIVE proteinuria, with reduced GFR and POOR response to corticosteroids
    • also high incidence of hematuria/HTN (MIXED)

P: possibly progression from Minimal Change Disease, but with extra epithelial damaged and sclerosis
- due to mutations in SLIT DIAPHRAGM and podocyte CYTOSKELETAL structure

21
Q

Focal Segmental Glomerulosclerosis

What are the 5 possible mutations that can be seen in this disease? (N/P/AA/T/A)

A
  1. Nephrin Gene - collapse of filtration barrier
    • Chromosome 19q13
  2. Podocin - steroid resistant pediatric form
    • Chromosome 1q25-q31; Auto RECESSIVE
  3. Alpha-actinin 4 - insidious, high rate of RI
    • Auto DOMINANT
  4. TRP6 - adult onset FSGS
  5. Apolipoprotein L1 (APOL1) - inc. risk in African Amer.
    • Chromosome 22
    • inc. resistance to TRYPANOSOME infection
22
Q

Focal Segmental Glomerulosclerosis

What is its morphology, what two molecules are found in sclerotic areas, and how does it present clinically?

What three viral illness is it commonly associated with?

A

M: minority of glomeruli have focal/segmental lesions (see eosinophilic hyaline) with sclerosis; FOAM CELLS usually present; effacement of podocytes
- see IgM and C3 in sclerotic areas

C: significantly dec. GFR with azotemia; hypertensive

  • AA kids/adults with chronic viral illnesses
  • does NOT respond to corticosteroids
  • transplant or dialysis is INEVITABLE

chronic viral illnesses = HIV, Hep B, Hep C

23
Q

What is Collapsing Glomerulosclerosis and what is it frequently associated with?

What is its characteristic feature?

A

CG: distinct variant of FSGS with retraction/collapse of ENTIRE glomerular tuft and is associated with tubular injury = MICROCYSTS
- CF = prolif/hypertrophy of glomerular visceral epi cells

  • *most characteristic lesion of HIV NEPHROPATHY**
    • lots of tubuloreticular inclusions in endothelial cells that are modifications of ER in cells by circulating IFN-a
    • NOT found in idiopathic FSGS = diagnostic for Dx
24
Q

What are patients with HIV nephropathy most at risk of infection by?

A

Streptococcus pneumonia

  • loss of immunoglobulins in urine inc. susceptibility to STAPH and STREP infections (acute pyogenic)
25
Q

Membranoproliferative Glomerulonephritis

What is it also known as and what is it?

A

aka Mesangiocapillary Glomeruloneprhitis

  • not a specific disease, but an immune-mediated injury due to immune deposition
  • see mesangial interposition = proliferation of mesangial cells into GBM = “tram track” appearance
26
Q

Membranoproliferative Glomerulonephritis

What is the difference between MPGN Type 1 (2 subsets) and MPGN Type 2?

A

Type 1: MIXED nephrotic/nephritic; IgG + C3/C1q + C4

  • subENDOTHELIAL deposits
  • Primary - KIDS (50% get chronic renal failure)
  • Second - ADULTS (Hep C, SLE, CCL, lymphomas)

Type 2: ALTERNATE complement activation; IgG + C3

  • DENSE DEPOSITS (C3 ONLY in GBM, not deposits)
  • C3 Nephritic Factor = C3 convertase always ON
  • sicker, nephritic patients

HYPOCOMPLIMENTEMIA

27
Q

Membranoproliferative Glomerulonephritis (Type 1)

What is its pathogenesis, what does it look like morphologically, and and how does it present clinically?

A

P: immune complex deposition and activation of CLASSICAL and ALTERNATIVE complement pathways
- LOW C3; mesangial proliferation

M: hypercellularity of mesangium and capillaries (“double contour” or “tram track” appearance)
- granular subENDOTHELIAL deposits

C: Kids = nephrotic syndrome w/nephritic component; Adults = Chronic Immune Complex disorder

28
Q

Membranoproliferative Glomerulonephritis (Type 2)

What is its pathogenesis, what does it look like morphologically, and how does it present clinically?

A

P: excessive activation of ALTERNATIVE complement pathway in pts. with C3 nephritic factor (C3NeF) autoantibodies (protect C3 convertase from turning off); normal C1/C4 lvls (not involved in this)

M: INTRAMEMBRANOUS deposits; permeation of GBM lamina densa by extremely electron dense ribbon of material; C3 around, NOT IN, dense deposits
- mesangial proliferative pattern (“tram track”)

C: poor prognosis; 50% –> ESRD; reoccurs in 90% of transplant recipients

29
Q

IgA Nephropathy

What is it and what is its pathogenesis?

What is it the most common cause of worldwide?

A
  • primary renal disease (Berger) with IgA deposits in the MESANGIUM presenting with RECURRING hematuria (MCC of glomerulonephritis WORLDWIDE)
    • Henoch-Schonlein Purpura is SYSTEMIC version

P: associated with IgA1 mutation; liver normally decomposes polymeric IgA, so disease can be seen in pts. with LIVER DISEASE

  • complexes activate ALTERNATIVE complement pathway (no C1q/C4)
  • see inc. in pts. dealing with MUCOSAL INFECTIONS
30
Q

IgA Nephropathy

What is its morphology and how does it present clinically?

A

M: mesangial proliferation/widening; diagnosis based on IF stains for complement or IgA in GRANULAR mesangial pattern (low IgG/IgM)

C: typically seen in patients presenting with GROSS HEMATURIA following an infection of the respiratory/GI tract
- bleeding will last a few days, then come back every few months (RECURRENT HEMATURIA)

31
Q

What are the two types of Hereditary Nephritis and what causes them?

A
  1. Alport Syndrome
  2. Thin Basement Membrane Lesion
  • caused by mutations in collagen genes that manifests primarily as glomerular injury
32
Q

What is the mneumonic for Alport Syndrome?

A 
A - Alport Syndrome ("A is for Alfred")
LP - records (i.e you can't hear shit = DEAFNESS)
   - NOT seen in TBML
O - ocular problems
   - NOT seen in TBML
R - renal failure
   - NOT seen in TBML
T - THICKENING of basement membrane and Type 4 collagen
   - membrane THINNING in TBML
33
Q

Alport Syndrome

What is it, what is its pathogenesis, what does it morphologically look like, and how does it present clinically?

A
  • X-linked disorder involving collagen formation (females just have hematuria, but males express full syndrome with ERSD by 40 years of age = POOR PROGNOSIS)

P: defect in Type 4 collagen = defective GBM
- collagen crucial for function of GBM, eye lens, and cochlea; patients are IMMUNE to Goodpastures

M: alternating thickening/thinning glomerular basement membrane (“Basket Weave” or “Moth eaten”)
- stain for Alpha chains is negative in these pts.

C: gross or microscopic hematuria; red cell casts; vision and auditory abnormalities

34
Q

Thin Basement Membrane Disease (Benign Familial Hematuria)

How does it present, how is the diagnosis confirmed, and what is it caused by?

A
  • very common, asymptomatic hematuria with NORMAL renal function = EXCELLENT PROGNOSIS

Dx: confirmed with EM showing DIFFUSE THINNING of GBM; NO IgA immune deposition (vs IgA Nephropathy)

  • due to defect in Type 4 collagen; alpha-5 Type 4 collagen is present with NO ocular/auditory lesions
35
Q

Diffuse Lupus Nephritis (Class 4)

What is it and what does it affect in the kidneys?

A
  • Lupus forms Ab-Ag complexes that deposit in the glomerular filtration barrier; MO activation leads to injury and eventual fibrosis
    • “Wire Loop” appearance

Class 4: MOST COMMON and severe form of Lupus

  • involvement of > 50% of glomeruli
  • lateral crescents that fill Bowman’s Capsule
  • subENDOTHELIAL deposits with GRANULAR IF
36
Q

Henoch-Schonlein Purpura

What is its morphology, what two things is it associated with, and how does it differ from Berger Disease?

A

M: Dx: made by IgA deposited in the renal mesangium, sometimes with IgG/C3 that can extend into the capillary loops

  • see with PRURITIC skin rashes on extensor surfaces of arms/legs and buttocks
  • onset in first 3-8 yrs of life with EXCELLENT prog.
  • associated with Atopy and URI
  • Berger is LOCALIZED, HSP is SYSTEMIC
37
Q

Fibrillary Glomerulonephritis

What does it look like, what does it NOT stain with, and how does it present clinically?

A
  • fibrillar deposits in the mesagnium and glomerular capillary walls (looks like Amyloid fibrils)
    • NO CONGO RED STAINING
  • selective deposition of polyclonal IgG (IgG4), IgK/IgL light chains, and C3
  • mixed nephrotic/nephritic syndrome
38
Q

Essential Mixed Cryoglobulinemia and Glomerulonephritis

What is it caused by, what is it associated with, and what does it lead to?

A
  • IgG-IgM complexes (cryoglobulins) induce cutaneous vasculitis, synovitis, proliferative glomerulonephritis (MPGN TYPE 1)
  • associated with Hepatitis C –> MPGN Type 1

CPR II Exam 3 (12 decks)

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