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Renal > Nephritis > Flashcards

Nephritis Flashcards

1
Q

What is the distinguishing feature in nephritic syndromes?

A

hematuria with or without mild proteinuria

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2
Q

In addition to hematuria, nephritic syndromes are characterized by what findings?

A
  • dysmorphic RBCs
  • red cell casts
  • oliguria
  • azotemia
  • HTN
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3
Q

What is the common pathophysiologic denominator in all nephritic diseases?

A

proliferation of cells within the glomerulus, including leukocyte infiltration. These inflammatory cells damage the GBM allowing RBCs to escape into urine, resulting in attempts to compensate: oliguria and hypertension. The GBM damage may be enzymatic or structural (by immune complexes), and damage to other components (such as the endothelium and epithelium) will give rise to associated mild proteinuria.

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4
Q

Acute post streptococcal glomerulonephritis is a sub type of which disease?

A

diffuse proliferative glomerulonephritis

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5
Q

What does the word “proliferative” mean in diffuse proliferative GN?

A

neutrophilic infiltrate of the glom

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6
Q

” Classically refers to hematuria in a young child 7-30 days after an infection, a transient upper respiratory (URI) or skin infection by Group A β-hemolytic Strep. “

What are the LM findings for this condition?

A

PSGN

LM: neutrophils and monocytes infiltrating the glom with scattered fibrin thrombi

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7
Q

” Classically refers to hematuria in a young child 7-30 days after an infection, a transient upper respiratory (URI) or skin infection by Group A β-hemolytic Strep. “

What are the FM findings for this condition?

A

PSGN

FM: granular IgG and C’ deposits,

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8
Q

” Classically refers to hematuria in a young child 7-30 days after an infection, a transient upper respiratory (URI) or skin infection by Group A β-hemolytic Strep. “

What are the EM findings for this condition?

A

PSGN

dense subepithelial humps

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9
Q

What kind of immune complexes are deposited in PSGN?

A

IgG and C’ on the GBM

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10
Q

What is RPGN?

A

RPGN is a clinical syndrome of rapid loss of renal function,

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11
Q

Describe crescentic GN. What is this a histologic sign for?

A

crescents in most gloms: proliferation of parietal epithelial cells of Bowman’s capsule, needs to involve >50% of gloms (since may have occasional crescents in other types of kidney injury).

RPGN sign

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12
Q

Do RPGN patients require dialysis?

A

Yes, within weeks or months

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13
Q

What findings divide crescentic GN into 3 types?

A

FM and EM findings

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14
Q

How many types of crescentic GN are there?

A

3

  1. anti GBM
  2. IC
  3. pauci-immune
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15
Q

This disease has linear IgG deposits +/- C3 along with crescentic GN on FM and EM.

A

Type I anti GBM RPGN

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16
Q

How is type I anti GBM RPGN treated?

A

plasmapharesis to remove anti GBM antibodies

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17
Q

granular IC deposits with ‘lumpy-bumpy’ appearance on EM with crescentic GN.

This is a sign of what disease?

A

type II IC RPGN

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18
Q

How does type II IC RPGN occur?

A

as complication of any of the autoimmune disorders with immune complex formation (such as SLE, post-streptococcal GN, or Henoch-Schonlein purpura)

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19
Q

Crescentic GN without anti-GBM antibodies, and FM/EM negative, as well as +ANCAs is what disease?

A

type III pauci immune RPGN

20
Q

What causes type III pauci immune RPGN

A

complication of vasculitis (polyarteritis nodosa or granulomatosis with polyangiitis or eosinophils).

21
Q

What are the 3 main types of ANCA?

A

MPO (p ANCA)
PR 3 (c ANCA)
atypical (x ANCA)

22
Q

What histological type of crescentic GN/RPGN is associated with + ANCA?

A

type III pauci immune

23
Q

What are the 2 classic vasculitides assocaited with crescentic GN and therefore, RPGN?

A

MPA: Microscopic polyangiitis = vasculitis with neutrophils (usually p-ANCA)
GPA: Granulomatosis with Polyangiitis = formerly Wegener’s granulomatosis – vasculitis with neutrophils and granulomas (usually c-ANCA)

24
Q

What is microscopic polyangitis?

A

vasculitis with neutrophils (p ANCA) that can cause type III crescentic GN and RpGN

25
Q

What is granulomatosis with polyangitis?

A

Wegener’s granulomatosis - vasculitis with neutrophils and granulomas (c ANCA). Can cause type III pauci immune crescentic GN and RPGN

26
Q

What is Eosinophilic Granulomatosis with Polyangiitis ?

A

A cause of crescentic GN and RPGN. vasculitis with eosinophils and granulomas (usually p-ANCA, seen in 40% of cases)

27
Q

What syndrome is characterized by recurrent hematuria in kids and young adults? First episode occurs after an URI or other infection; however, the hematuria recurs irregularly May be gross or microscopic.

A

IgA nephropathy aka Berger disease

28
Q

What nephritic syndrome is a variant of Henoch Schonlein purpura?

A

IgA nephropathy

29
Q

What is the LM finding for IgA nephropathy?

A

variable with focal glomeruli affected with proliferative mesangium or crescents

30
Q

What is the FM finding for IgA nephropathy?

A

granular IgA deposits in the mesangium with C3, may have some IgG and IgM

31
Q

What is the EM finding for IgA nephropathy?

A

electron-dense deposits in the mesangium

32
Q

What guides treatment in lupus nephritis?

A

the classification/grading

33
Q

What are active lesions in lupus nephritis?

A

hypercellularity; endocapillary fibrin, and apoptotic bodies

34
Q

What are chronic changes in lupus nephritis?

A

sclerosis (scarring) and crescents.

35
Q

What FM finding is characteristic for lupus nephritis?

A

“Full house” on FM (including IgG, IgM, and IgG with C3 and C1q) is a characteriAcustic feature of lupus nephritis.

36
Q

What disease is positive for apple green birefringence on congo red stain?

A

amyloidosis

37
Q

How is amyloidosis classified?

A

chemically, by protein type

38
Q

What type of nephritic syndrome may present with pulmonary involvement? (i.e. hemoptysis)

A

anti GBM type 1 RPGN

39
Q

What kind of nephritic syndrome is associated with vasculitis?

A

type III RPGN

40
Q

What is the current thought regarding mechanism of hematuria in nephritic syndromes?

A

Enzymatic or mechanical damage to the GBM, which allows RBCs to squeeze out into the filtrate.

41
Q

You encounter a young kid with hematuria; how do you differentiate acute post-streptococcal GN from IgA nephropathy?

A

Acute post-streptococcal GN will have neutrophils and fibrin thrombi (LM), IgG+C’ (FM), and subepithelial deposits (EM), whereas Berger disease will have crescents and increased mesangial cells (LM), granular IgA (FM), and mesangial deposits (EM).

42
Q

What is a key histologic feature of all 3 RPGN?

A

Crescents.

43
Q

What is ANCA?

A

ANCA is anti-neutrophil cytoplasmic antibody, usually directed against MPO or PR3, and associated with certain systemic vasculitides – GPA, EGPA, and MPA. Biopsies in patients with RGPN due to vasculitis will have negative FM.

44
Q

What is the significance of the classification system of Lupus nephritis?

A

The classification system of renal injury in Lupus provides a staging and descriptive tool used to guide treatment. The system consists of 6 classes progressing from minimal damage to end-stage damage.

45
Q

What is amyloid and how does it cause damage to organs?

A

Amyloid is deposition of various proteins capable of forming beta-pleated sheets in a crystalline structure, generally involves filtration regions of the body – basement membranes of various organ systems including lung, kidney, skin, GI tract, bladder, etc. Deposition of this substance usually interferes with normal function of the organ, preventing filtration, reabsorption, or impeding vascular compliance, causing signs and symptoms (disease called amyloidosis). Classified and treated based on the underlying type of protein.

Renal (28 decks)

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