Nephritic Syndrome Flashcards

(43 cards)

1
Q

What 2 types of Nephritic Syndrome Fall under the category of:
Visceral Epithelial Injury: Podocyte Disorder?

A
  • Minimal Change Disease

* Focal Segmental Glomerulosclerosis

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2
Q

What 4 types of Nephritic Syndrome Fall under the category of:
Immune Complex Formation?

A
  • Membranous Nephropathy (subepithelial) - really this is a nephrotic syndrome
  • Post-infectious Glomerulonephritis (subepithelial)
  • Membranoproliferative Glomerulonephritis (SubENDOthelial)
  • IgA nephropathy (mesangial)
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3
Q

What 3 types of Nephritic Syndrome Fall under the category of:
Glomerular Basement Membrane Disease

A
  • Anti-glomerular Basement Membrane Disease
  • Alport’s Syndrome
  • Thin Basement Membrane
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4
Q

What 2 types of Nephritic Syndrome Fall under the category of:
Vascular Injury Syndromes?

A
  • ANCA-associated glomerulonephritis/pauci-immune glomerulonephritis
  • Hemolytic Uremic Syndrome (HUS) and Thrombotic Thrombocytopenia Purpura (TTP)
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5
Q

What are some important Side Effects seen with most Nephritic Syndromes?

A

Hematuria
• microscopic or macroscopic
• Red Blood Cell Casts

HTN
Oliguria
Edema (moderate)
Mild to moderate proteinuria
Azotemia
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6
Q

Where are the immune deposits located in:
• Membranoproliferative GN
• IgA nephropathy

A

Membranoproliferative GN = SUBENDOTHELIAL

IgA Nephropathy = MESANGIAL

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7
Q

What are 4 diseases associated with SubENDOthelial and mesangial immune DEPOSITS?
• what particle/process is causing damage in these diseases?

A
  • MEMBRANOPROLIFERATIVE GN
  • IgA Nephropathy (mesangial)
  • Lupus Nephritis
  • Post-Infectious GN

*Diseases are associated with circulating immune complexes that deposit on the basis of size, charge, etc.

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8
Q

What are some diseases that cause actual ENDOTHELIAL CELL INJURY?
• how?

A
  • Thombotic Microangiopathies

* Immune complexes/PARAproteins

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9
Q

What is the most common causes of Paraprotein entrapment that leads to Endothelial cell injury?

A

Multiple Myeloma

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10
Q

What causes endothelial damage nephritic syndrome?

A

INFLAMMATORY RESPONSE that results from…

CYTOKINES and Autacoids are released which UPREGULATE Adhesion Molecules on Endothelial and circulating immune cells

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11
Q

T or F: HEMATURIA is the clinical indication that nephritic syndrome is occuring.

A

True

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12
Q

What are the two forms of Membranoproliferative Glomerulonephritis?
• Prevalence
• Deposition location?

A

Type I: MOST COMMON

Type II: Dense Deposit Disease

Deposition Location: SubENDOthelial

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13
Q

Membranoproliferative Glomerulonephritis Type I
• light microscopic changes
• KEY thing to recognize visually
• Important Lab Value

A

Light Microscopic Changes:
• Hypercellularity of Glomerular Tuft
• Increased mononuclear cells within the expanded mesangium and in capillary lumens

KEY:
• Tram-Track Appearance

Lab Value:
• Reduced C3 levels

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14
Q

What is frequently the cause of Membranoproliferative Glomerulonephritis Type I in adults?

A

Hepatitis C

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15
Q

How do people of all ages typically present with MPGN (membranoproliferative glomerulonephritis) type I?

A
  • Microscopic Hematuria
  • Nephrotic range proteinuria and Nephrotic Syndrome is possible
  • Acute Nephritic Syndrome
  • Rapidy progressive glomerulonephritis (PRGN)
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16
Q

What is the typical prognosis of MPGN type I?

• treatment?

A

Px: USUALLY - slow progression to end stage renal disease; RARELY spontaneous remission may occur

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17
Q

Dense Deposit Disease (Membranoproliferative glomerulonephritis type II).
• KEY lab terms (pathophysiology)
• KEY TERM for histology

A

Key Lab Terms:
• LOW C3 is often present because 80% of ppl. ohave C3 NEPHRITIC FACTOR which binds and stabiliizes C3

Key Histo Terms:
• Dark SubENDOthelial ribbons on Electron Microscopy (aka electron dense deposits)

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18
Q

What causes Tram Track appearance?

• associated disease?

A

Tram Tracks:
• Splitting of the GBM is associated with Tram Track Appearace is due to MESANGIAL cells (which have macrophage-like function) PENETRATE the Basement Membrane in pursuit of Immune Complexes

19
Q

What does the glomerulus look like in Membranoproliferative Glomerulonephritis and Dense Deposit Disease?
• Basement Membrane
• Cells
• Overall Appearance

A

Glomeruli = Large and Lobular

  • All Cell Types have proliferated - Mesangial, Endothelial, and Leukocytes
  • GBM has thickened
20
Q

What differences exist between MPGN type I and II on immunofluorescence?

A

MPGN type I:
• Granular C3 positive Basement Membrane

Dense Deposit Disease:
• BRIGHT C3 DEPOSITS in MESANGIUM and some in the capillary wall

21
Q

T or F: in some cases MPGN can be associated with MONOCLONAL GAMMOPATHIES

22
Q

MPGN vs. DDD:

• Pathophysiology

A

MPGN:
Immune Complexes in GBM induced CLASSICAL COMPLEMENT PATHWAY*

Immune Complexes in GBM induce ALTERNATIVE COMPLEMENT PATHWAY

• this leads to damage endothelial cellls and basement membrane by leukocyte recruitment

23
Q

What is the Most Common Primary GN worldwide?

• what are some secondary causes of this disease?

A

• IgA Nephropathy - Berger Disease

Secondary Causes: 
• Henoch - Schoenlein Purpura
• Ankylosing Spondylitis 
• Dermatitis Herpetiformis
• Celiac Disease
• Inflammatory Bowel Disease
• Cirrhosis 
• Psoriasis
24
Q

What is the location of Immune Deposits in IgA nephropathy?

• aka what is the pattern of Injury

A

IgA containing Immune complexes are found mostly in the MESANGIUM

Pattern of Injury:
• Mesangioproliferative Pattern of Injury

25
What is Classically the trigger of IgA nephropathy?
IgA nephropathy: • Often Triggered by Upper Respiratory Tract infection ***IMPORTANT: sediment forms in a SYNPHARYNGITIC manner i.e. within 1 to 2 days of infection (not after infection)****
26
What is the problem with the IgA formed in IgA nephropathy (Berger's disease)? • Where do these immune complexes form? • why do they form?
GALACTOSE DEFICIENT IgA1 circulating immune complexes deposit in the MESANGIUM*** WHY: • underglycosylation of O-linked glycans that terminate with N-acetylgalactosamine instead of galactose • OTHER IgG or IgA antibodies recognize this unglycosylated region and form IMMUNE COMPLEX
27
How does IgA nephropathy present?
SUPER VARIABLE: * Asymptomatic * Microscopic Hematuria * Intermittent Gross Hematuria * Synpharyngitic Hematuria * Nephrotic (15%) or non-nephrotic proteinuria * Acute Glomerulonephritis * Rapidly Progressive Glomerulonephritis
28
T or F: patients must have elevated to serum IgA levels to have IgA nephropathy.
FALSE, only ~50% of patients with this have increased serum IgA levels
29
What are some Poor Prognostic Indicators of IgA nephropaty?
* Heavy Proteinuria * Decreased GFR at onset of disease * Older age at Onset * Uncontrolled HTN * Cresents and/or tubulointerstitial fibrosis/atrophy
30
T or F: immune deposits are found in the subendothelium in IgA glomerulonephropathy
FALSE, IgA nephropathy has Immune Deposits in the MESANGIUM
31
What two diseases involve the basement membrane?
* Anti-GBM disease | * Hereditary Nephritis
32
What causes anti-GBM disease? • what is another name for this disease? • Disease progression?
Anti-GBM Cause: • IgG Autoantibodies against a noncollagenous portion of the ALPHA-3 SUBUNIT of type IV collagen Development: • Disease is rapidly progressing!!! - days to weeks until these people develop renal failure
33
What is the appearance of anti-GBM antibodies in immunofluorescence? • Appearance on H and E? • correlation between concentration of anti-GBM in the membrane and disease activity.
Appearance: • LINEAR pattern on Immunofluorescence - PATHOpneumonic for Goodpasture's • H and E = CRESCENT formation (focal or diffuse); Glomerulonephritis Anti-GBM conc.: • NO correlation between anti-GBM titers and disease activity
34
What other antibody besides Anti-alpha-3 collagen antibodies are present in Goodpastures?
MPO-ANCA
35
What besides renal dysfunction, what are some other symptoms/problems associated with Goodpastures?
Pulmonary Problems: • Cough, dyspnea, crackles, hemoptysis (may precede or coincide with renal dysfunction) • Smoking makes this worse Blood Problems: • Anemia out of proportion to renal insufficiency • NORMOTENSIVE with decreased renal function (late complication) Bone/joint Problems: • Arthritis/Arthralgias
36
What is the treatment for Goodpasture's (anti-GBM) disease?
* Corticosteroids * Plasmapheresis * Cytotoxic Agents
37
What happens to the glomerulus in light of the formation of crescents in Goodpasture's syndrome?
• Glomerular Tuft is compressed by PROLIFERATING EPITHELIAL CELLS in the crescent
38
What is the progression of disease severity in crescent formation? • when can you classify a disease as a crescentric glomerulonephritis?
Segmental Proliferative and Necrotizing Lesions => Cellular Crescents => Fibrocellular Crescents => Fibrous Crescents Classification: • Referred to as a diffuse crescentric glomerulonephritis if greater then 50% glomeruli are involved under light microscopy
39
What is the etiology of crescent formation in the kidney?
• May be due to severe damage ot capillary walls that tear and necrose the GBM Result: • RBCs, WBCs, fibrinogen, and plasma constituents enter Bowman's space and cause proliferation of mononuclear cells and parietal epithelial cells
40
In Rapidly progressive glomerulonephritis what is seen on Immunofluorescence?
* FIBRINOGEN stains into GREEN CRESCENTS
41
Alport Syndrome • Cause? • Who is most often affected? • Associated Symptoms?
Cause: • X-linked (most often) defects in ALPHA-5 COLLAGEN TYPE IV Who is affected: • Because its X-linked MALES are most often affected Associated symptoms: • Sensorineural Hearing Loss • Lens abnormalities, Platelet Defects • Esophageal Leiomyomas
42
Alport Syndrome • Key terms • microscopy used in dx?
BASKET WEAVE APPEARANCE EM is where you see basket weave appearance, but early in the disease you don't see anything
43
Thin Basement membrane Disease • cause • severity of Disease • appearance
Cause: • Defects in ALPHA-3 or ALPHA-4 collagen type IV Severity: • Heterozygotes = Benign • Homozygotes/Compound Heterozygotes = Affected Appearance: • GBM appears at 1/2 thickness