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Renal > 7 - Glomerular Disease 1 > Flashcards

7 - Glomerular Disease 1 Flashcards

1
Q

What are three mechanisms of glomerular disease?

A
  1. Immune complex deposition: circulate and deposit in the glomerulus; activates complement causing PMN chemotaxis
  2. Antibodies against the glomerular basement membrane (GBM) or glomerular antigens
  3. Cytokine production by inflammatory cells
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2
Q

Describe diffuse, focal, global, and segmental in terms of glomerular disease?

A

Diffuse is when it impacts many glomeruli.

Focal is when it impacts less than 50% of the glomeruli.

Global means it impacts the entire glomerulus.

Segmental means it impacts a segment of a glomerulus.

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3
Q

How does glomerular disease present?

A

Any of these things at any time:

  • loss of GFR
  • hematuria - quality
  • proteinuria - quantity
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4
Q

NephrOtic syndrome is a constellation of what things?

A
  • Proteinuria >3.5 g/day
  • Hypoalbuminemia
  • Edema
  • Hyperlipidemia
  • Lipidemia
  • Hypercoagulability
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5
Q

Nephritis is a constellation of what things?

A
  • Mild proteinuria
  • Hematuria
  • Hypertension
  • Edema
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6
Q

What are the three primary diseases that cause nephrotic syndrome?

A
  1. Minimal change disease
  2. Focal segmental glomerulosclerosis
  3. Membranous nephropathy
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7
Q

What are some causes of acute glomerulonephritis?

A
  • IgA nephropathy
  • Post-infectious GN
  • Anti-GBM disease/Goodpasture’s
  • Small vessel vasculitis
  • Lupus nephritis
  • Membranoproliferative GN
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8
Q

What is IgA nephropathy? What age group gets it? What is seen?

A

Most common glomerulonephritis WORLDWIDE.

Most pts between 10 and 50

Hematuria, frequently in conjunction with a URI (Synpharyngitic hematuria) ; if proteinuria present it’s generally mild.

Many cases are subclinical.

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9
Q

What is seem on immunofluorescence in IgA nephropathy? What is seen on light micropscopy?

A

IF: Mesegangial IgA deposition

LM: variable mesangial hypercellularity

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10
Q

What is the prognosis in IgA nephropathy based on? How can it be treated?

A

Prognosis based on serum creatinine, BP, and degree of proteinuria - 40% develope chronic kidney disease.

Fish oil may slow progression.

ACE inhibitors to control high BP

Corticosteroids and other immunosuppressants may be used in progressive disease.

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11
Q

What is Henoch-Shonlein purpura? What are the manifestations?

A

Systemic disorder characterized by IgA deposition in multiple organs:

  • skin - non-blanching purpura on legs/buttocks
  • joints: transient arthralgias
  • GI: abd. pain, vomitting, melena
  • Kidney: hematuria (ie this is a nephritis!); rarely progresses to renal failure
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12
Q

What is post-infectious glomerulonephritis? When does it occur?

A

Post-strep GN is a classic example.

Follows infection of group A beta-hemolytic streptococcus. 7-14 days after after pharyngitis or 14-28 days after skin infection.

Sudden onset HTN, azotemia, oliguria (decreased urine), edema, and cola-colored urine.

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13
Q

What do lab tests show in post-strep GN?

A
  • Low C3 copmlement
  • Anti-streptolysin O (ASO) can be elevated
  • Urinalysis: RBCs, mild proteinuria
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14
Q

What is seen on IF and EM in post-strep GN?

A

IF: granular capillary wall and mesangial IgG and C3

EM: mesangial and large subepithelial “hump-like” deposits

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15
Q

What is the prognosis of post-strep GN?

A

95% of children will recover with conservative management (~1% will progress to renal failure)

60% of adults will recover promptly.

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16
Q

What is rapidly progressive glomerulonephritis? What are some causes?

A

Classic nephritis syndrome with rapid progression (days to weeks) to renal failure.

Somtimes referred to as “crescent GN”

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17
Q

What are some causes of rapidly progressive glomerulonephritis?

A
  • Anti-GBM/Goodpasture’s
  • Immune complex GN: lupus nephritis, post-infectious, cryoglobulinemia
  • ANCA associated GN (Pauci immune)
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18
Q

How does goodpasture’s syndrome present? Who gets it?

A

Males > females

May present as pulmonary-renal syndrome with hemoptysis, pulmonary infiltrates, and glomerulonephritis.

Due to circulating anti-GBM antibody an antigen in the apha3 chain of type IV collagen.

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19
Q

How would you diagnose goodpastures syndrome (anti-GBM)? How do you treat it?

A

+ anti-GBM antibody in the blood

LINEAR IgG and C3 on kidney biopsy

EMERGENT treatment with plasmapheresis, prednisone, and cytoxan (immunosuppressant).

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20
Q

What causes pauci-immuno GN?

A

Crescenteric GN with little (pauci means little) deposition of immune reactants

Idiopathic OR associated with antineutrophil cytoplasmic antibody (ANCA) vasculitis.

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21
Q

What are examples of small vessel vasculitis that cause pauci-immune GN?

A
  1. Microscopic polyangitis - no granulomatous inflammation and no asthma
  2. Granulomatosis with polyangitis - necrotizing granulomatous inflammation; no asthma
  3. Eosinophilic granulomatosis with polyangiitis - necrotizing granulomatous inflammation, asthma, eosinophilia
22
Q

What is granulomatous with polyangiitis (GPA)? How does it present and what does a biopsy show?

A

Granulomatous vasculitis of medium to small arterioles.

c-ANCA + in 80%

Presents with upper resp tract symptoms (sinusitis), mononeuritis multiplex, purpura, and nephritis.

Renal biopsy shows cresenteric GN without immune deposits.

23
Q

What is the seen clinically, on light microscopy, and on IF with Anti-GBM/goodpastures?

A

Clinically: +anti-GBM antibody in blood

LM: crescenteric GN

IF: Linear IgG and C3

24
Q

What is the seen clinically, on light microscopy, and on IF with rapidly progressive GN from immune complexes?

A

Clinically: lupus or post-strep

LM: crescenteric GN

IF: variable deposition of IC and cmoplement

25
Q

What is the seen clinically, on light microscopy, and on IF with pauci-immune rapidly progressive GN?

A

Clinically: ANCA+

LM: crescenteric GN

IF: negative

26
Q

What primary renal diseases can cause nephrotic syndrome? Which most commonly occurs in children?

A
  • Membranous nephropathy
  • Focal segmental glomerulosclerosis (FSGS)
  • Minimal change disease

In children ~80% will have minimal change disease

27
Q

What are secondary causes of nephrotic syndrome?

A

Systemic disease:

  • diabetes mellitus
  • SLE
  • amyloidosis
  • Infections: HIV, HepB, HepB, syphilis
  • Drugs: NSAIDs, gold, penicillamine
28
Q

How do you diagnose nephrotic syndrome?

A

Helpful lab studies for the secnodary causes.

Renal biopsy generally indicated.

29
Q

What is the treatment for all causes of nephrotic syndrome?

A

ACE inhibitor or ARB to lower intraglomerular pressure and reduce proteinuria.

Lipid lowering therapy (statins)

Diuretics and salt restriction to improve edema

30
Q

What is the most common cause of nephrotic syndrome in children? What is the peak incidence? What is the treatment and prognosis?

A

Minimal change disease

Peak incidence ages 2-6, 5% progress to ESRD.

Spontaneous remissions can occur; treat with steroids often induces remission but 75% relapse (fewer relapses after puberty).

31
Q

What can cause minimal change disease in adults?

A

Idiopathic or associated with:

  • Drugs: NSAIDs
  • Neoplasms such as Hodgkin’s lymphoma
  • Infections such as syphilis or HIV
32
Q

What is seem on LM and EM in minimal change disease?

A

LM: glomeruli, interstitium and tubules appear normal

EM: podocyte foot process effacement (fusion)

33
Q

What is the treatment of minimal change disease in adults and children?

A

Children respond well to corticosteroids

Majority of adults respond to steroids: usually takes longer than in children; partial remission may occur.

34
Q

Who gets membranous nephropathy and what is it caused by?

A

Most common cause of nephrotic syndrome in caucasion adults.

Caused by antibodies to the podocyte antigens phospholipase A2 receptor (70%) or thrombospondin type-1 domain containing 7A (10%).

35
Q

What are secondary causes of membranous nephropathy?

A

HepB infection

SLE

Neoplasms: consider age-appropriate cancer screening

Drugs: gold, NSAIDs, murcury, captopril, penicilliamine

36
Q

Describe the onset and symptoms seen in membranous nephropathy?

A

Onset is generally insidious.

Pts usually present with heavy proteinuria and nephrotic syndrome.

Renal vein thrombosis occurs in ~20%.

37
Q

What is seem on LM, IF, and EM in membranous nephropathy?

A

LM: diffuse thickening of the GBM, and GBM “spikes” on silver stain

IF: granular GBM deposits of IgG

EM: subepithelial deposits

38
Q

What is the outcome of membranous nephropathy?

How are pts managed?

A

Rule of thirds:

  • 1/3 spontaneous remission
  • 1/3 partial remissions with stable function
  • 1/3 slowly progressive loss of renal function

Pts without poor prognostic factors managed conservatively with ACE-I or ARBs and followed closely. Otherwise +/- immunosuppressive drugs.

39
Q

What is focal segmental glomerulosclerosis (FSGS)? How does it compare to minimal change disease?

A

Most common cause of idiopathic nephrotic syndrome in african americans.

More aggressive than minimal change disease:

  • HTN, hematuria more common
  • renal dysfunction commonly progressive
  • ESRD occurs 5-20 yrs post-presentation
40
Q

What are primary, secondary, and hereditary focal segmental glomeruloscleoris (FSGS)?

A

Primary: usually presents with acute onset of nephrotic syndrome

Secondary: slowly increasing renal insufficiency and proteinuria

Hereditary: mutations in proteins that make up the glomerular slit diaphragm

41
Q

What are secondary causes of FSGS?

A

HIV

Obesity

Drugs: NSAIDs, herion

42
Q

What is seen on LM, IF, and EM in focal segmental glomerulosclerosis (FSGS)?

A

FM: focal and segmental glomerular sclerosis with capillary collapse

IF: negative or IgM and C3 in mesangium

EM: podocyte foot process effacement

43
Q

What does the prognosis of focal segmental glomerulosclerosis correlate with? What is the treatment? How often does it progress to ESRD?

A

Prognosis correlates with the degree of proteinuria

ACE inhibitors reduce the proteinurea

Corticosteroids can induce remission in some; difficult to treat steroid-resistant pts who relapse (immunosuppresives used)

Progression to ESRD in 50% at 10 yrs.

44
Q

What dglomerular diseases can cause nephrotic and nephritic features?

A

Membranoproliferative glomerulo-nephritis (MPGN)

Systemic lupus erythematous (SLE)

45
Q

What is the presentation of someone with membranoproliferative glomerulo-nephritis (MPGN)?

A

Proteinuria and hematuria commonly coexist

HTN occurs in 1/3

Low C3 complement is prominent feature

Variable clinical presentation

  • half have nephrotic syndrome
  • 30% asymptomatic proteinuria +/- hematuria
  • 20% acute GN
46
Q

What are some causes of secondary membranoproliferative glomerulo-nephritis (MPGN)?

A

Hepatitis C virus

SLE

Cryoglobulinemia

Neoplasms

47
Q

What is seen on IF and EM in membranoproliferative glomerulo-nephritis (MPGN)?

A

IF: granular C3 deposition

EM: subendothelial deposits

48
Q

What are characteristics in systemic lupus erythematosus (SLE)?

A

Multi-system autoimmune disorder

  • abnormal autoantibody production
  • immune complex deposition
  • inflammatory cell infiltration
49
Q

What is lupus nephritis and who gets it? How do you diagnose lupus nephritis?

A

Common cause of diffuse proliferative GN

40% of pts develop overt nephritis

Many of the clinical syndromes of renal disease can occur in the setting of SLE.

Renal biopsy important to classify the lesion in SLE (there’s 6 different classifications).

50
Q

What is the treatment for lupus nephritis?

A

General principal to manage any class:

  • aggressive BP control
  • control of lipid levels
  • appropriate treatment of extrarenal involvement
51
Q

How would you treat lupus nephritis classes III-IV? How common is renal failure in class IV?

A

Usually treated with corticosteroids + cytotoxic therapy

Class IV: renal failure rate 25% by 5-10 yrs.

Renal (19 decks)

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