Glomerular Diseases Flashcards

1
Q

glomerular diseases (or syndromes) are distinguished by: (4)

A
  • clinical presentation
  • urinalysis
  • amount of protein in urine
  • associations w/ other disease manifestations
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2
Q

nephrotic syndrome is characterized by:

A
  • > 3.5 g of protein in urine/ days (nephrotic range proteinuria)
  • hyperlipidemia
  • edema
  • hypoalbuminemia

*many pts w/ high proteinuria may not have the full syndrome

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3
Q

Conditions associated w/ nephrotic syndrome

A
  • focal segmental glomerulosclerosis
  • membranous glomerulopathy
  • minimal change glomerulopathy
  • DM neuropathy
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4
Q

focal segmental glomerulosclerosis

A
  • MC in african americans
  • diagnosis made by renal biopsy
  • tx: glucocorticoids and calcineurin inhibitors
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5
Q

membranous glomerulopathy

A
  • MC in white adults
  • may be associated w/ hep B, C, malaria, syphilis, malignancy, meds, tumors, lymphomas
  • diagnose by renal biopsy
  • search for the cause (could be infection or malignancy)
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6
Q

treatment of membranous glomerulopathy

A
  • up to 30% resolve spontaneously w/i 6 months
  • pts who persist after 6 mos or have thrombotic/embolic even should be treated:
  • glucocorticoids; cyclophosphamide; calcineurin inhibitors
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7
Q

what is the MC cause of nephrotic syndrome in children (and 10% of adults)?

A

minimal change glomerulopathy

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8
Q

secondary causes of minimal change glomerulopathy

A
  • meds: NSAIDs, lithium, pamidronate, interferons
  • infection (viral)
  • malignancy (thymoma, or hodgkin lymphoma)
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9
Q

tx of minimal change disease

A
  • treatment commonly works but relapse is typical

- glucocorticoids; cyclophosphamide; calcineurin inhibitors; rituximab

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10
Q

Diabetic nephropathy

A
  • MC cause of end stage kidney disease in US
  • renal bx no idicated unless suspicion of another glomerulopathy
  • tx: glycemic control
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11
Q

nephritic syndrome

A
  • inflammation of the glomerulus
  • hematuria
  • WBCs in urine
  • proteinuria
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12
Q

what is the hallmark in nephritic syndrome?

A

hematuria with the presence of dysmorphic RBCs in the urine w/ or w/o RBC casts

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13
Q

protein amount in proteinuria

A

-can vary from a small amount to the high levels like in nephrotic syndrome

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14
Q

3 pathophysiologic mechanisms in nephritic syndrome

A
  • anti-GBM antibodies
  • pauci-immune GN
  • immune complex deposition
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15
Q

pauci-immune GN is defined by what?

A

-necrotizing GN w/ few or no immune deposits

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16
Q

serums complement of the immune processes

A
  • anti-GBM and Pauci-immune: normal

- immune complex deposition: low levels

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17
Q

conditions that cause nephritis syndrome

A
  • rapidly progressive glomerularnephritis
  • focal segmental glomerulosclerosis
  • anti-GBM ab disease
  • pauci-immune GN
  • Immune complex mediated GN (IGAN) nephropathy
  • lupus nephritis
  • infection related GN (IRGN)
  • membranoproliferative GN (MPGN)
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18
Q

rapidly progressive GN (RPGN)

A
  • esp common in anti-GBM disease
  • renal failure occurs rapidly
  • may be associated w/ sx of systemic vasculitis (arthitis, epistaxis, hemoptysis, lung hemorrhage)
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19
Q

diagnosis of RPGN

A
  • serum tests may be positive for ANCA
  • may be positive for atibodies to anti-GBM
  • immunofluorescence microscopy on bx
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20
Q

tx of RPGN

A
  • all those except related to infection should get high dose glucocorticoids
  • usually also cyclophosphamide and or plasmaphoresis
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21
Q

focal segmental glomerulosclerosis (FSGS)

A
  • MC in african americans
  • secondar cause: DM, HTN, obesity, SS disease, HIV, drugs
  • diagnose w/ bx
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22
Q

tx of FSGS

A
  • only a few spontaneously resolve

- glucocorticoids and / or clacineurin inhibitors

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23
Q

anti-GBM antibody disease

A
  • autoimmune disease cause by antibodies directed against the noncollagenous domain of type IV collagen***
  • bx: crescentric GN***
24
Q

when would you see similar pathology to anit-GBM disease?

A

goodpasture’s syndrome

25
Q

diagnosis of anti-GBM disease

A
  • usually see all characteristics of nephritic syndrome
  • high anti-GBM antibodies
  • normal complement levels
  • proliferative nephritis w/ many crescents**
26
Q

tx of anti-GBM

A
  • immunosuppressive therapy

- cyclophosphamide and glucocorticoids combined w/ daily plasmapheresis

27
Q

pauci-immune GN

A
  • cause by microscopic vessel vasculitis affecting the glomerulus w/ few or no immune deposits
  • renal lesion can occur w/ or w/o a systemic vasculitits
  • most patients have circulating ANCA directed against neutrophils
28
Q

What are the 3 forms of vasculitis associated w/ Pauci?

A
  • granulomatosis w/ polyangitis (GPA)
  • microscopic polyangitis (MPA) - similar to GPA but w/o granulomas
  • eosinophilic granulomatosis w/ polyangitis
29
Q

GPA was formerly known as what?

A

Wegener’s

30
Q

eosinophilic granulomatosis w/ polyangitis was formally known as what?

A

Churg-Strauss syndrome

31
Q

kidney manifestations of Pauci

A
  • range from mild w/ some hematuria and proteinuria to RPGN
  • systemic sx may be mild: low grade fever, fatigue ext
  • could have systemic findings: leukocytoclastic vasculitis, pulm. dz, commonly have hs of asthma, pulmonary infiltrates, eosinophilia
32
Q

diagnosis of Pauci

A
  • more than 80% of GPA and MPA are ANCA positive

- normal complement levels

33
Q

tx of Pauci

A
  • glucocorticoids and cyclophosphamide w/ or w/o plasmaphoresis
  • rituximab in mild disease
34
Q

what is the MC cause of chronic GN?

A

IGAN

IgA Nephropathy

35
Q

manifestation of IGAN

A
  • chronic hematuria w/ or w/o proteinuria

- may also have external sites (Henoch-Shonlein purpura) or IGA vasculitis

36
Q

diagnosis of IGAN

A

renal bx

37
Q

treatment of IGAN

A
  • if mild not treated

- if dangerous: ACE inhibitors or ARBs, sometimes glucogorticoids

38
Q

lupus nephritis

A
  • prototypical immune complex nephritis

- immune deposits seen in all portions of the glomerulus

39
Q

clinical manifestations of lupus nephritis

A
  • external sx of SLE
  • active lupus serology (pos. ANA) (pos anti-daDNA)
  • decreased C3 C4 levels
  • massive classification of the histology
40
Q

tx of lupus nephritis

A

-for class 3 and 4 lesions, most pts benefit from combo immuno-suppressive therapy

41
Q

post-strep GN is now known as?

A
  • infection related glomerulonephritis (IRGN)

- b/c now it’s after staph and e.coli

42
Q

in IRGN, what is the timing difference b/w different bacteria?

A
  • strep: delayed 2 weeks

- staph and e.coli: simultaneous

43
Q

IRGN

A
  • immune complex medicated disease

- the antigen in the immune complex originates w/ the infectious organism

44
Q

clinical manifestation of IRGN

A

-usually present w/ acute nephritic syndrome

45
Q

diagnosis of IRGN

A

-usually requires bx to distinguish from other causes

46
Q

tx of IRGN

A

tx underlying infection

47
Q

membranoproliferative GN (MPGN)

A

-the pathology is in the mesangium and endocapillary proliferation along w/ thickening of the basement membrane

48
Q

diagnosis of MPGN

A

bx

49
Q

tx of MPGN

A

tx of the underlying pathology

50
Q

cryoglobulinemia

A
  • immune related proteins that precipiate at temps below 37 degrees
  • in vitro may be associated w/ a systemic inflammatory syndrome involving sm. - med. vessel vasculitis
51
Q

manifestations of cryoglobulinemia

A

can present w/ mild proteinuria to full blown nephrotic syndrome

52
Q

type 1 cryoglobulinemia

A

monoclonal IgG

53
Q

type 1 cryoglobulinemia was formerly known as what?

A

waldonstrom’s macroglobulinemia

54
Q

type II cryoglobulinemia

A

polyclonal IgG associated w/ hep C

55
Q

type III cryoglobulinemia

A

polyclonal IgG and IgM associated w/ infections, including hep C and autoimmune diseases