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*MHD Exam 5 > Nephrotic Diseases > Flashcards

Nephrotic Diseases Flashcards

1
Q

What is the hallmark of nephrotic syndrome?

A

heavy proteinuria due to increased permeability to plasma proteins

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2
Q

What are the diagnostic criteria for nephrotic syndrome?

A
  • massive proteinuria (>3.5 g or more daily protein loss in urine)
  • hyoalbuminemia (plasma albumin <3 g/dL)
  • generalized edema (most obvious clinical manifestation)
  • hyperlipidemia and lipiduria
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3
Q

Why does lipiduria happen in nephrotic syndrome?

A

It reflects the increased permeability of the GBM to lipoproteins.

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4
Q

What is the typical clinical presentation of membranous nephropathy?

A
  • edema
  • thrombosis (loss of ATIII)
  • infections
  • “foamy” (or frothy) urine
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5
Q

What is the typical patient population affected by membranous nephropathy?

A

young/middle aged adults 30-60 y/o

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6
Q

2nd most common cause of nephrotic syndrome

A

membranous nephropathy

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7
Q

What are possible causes of membranous nephropathy?

A
  • in-situ subepithelial immune complex formation
  • autoimmune response against renal antigen or SLE
  • carcinomas, leukemia, non-Hodgkin’s lymphoma
  • infections (malaria, Hep B, syphilis, schistosomiasis)
  • drugs (penicillamine, gold, mercury)
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8
Q

How do antibodies affect the glomerulus in membranous nephropathy?

A

They react with the basal surface of podocytes, causing injury to them (loss of slit diaphragms, foot process effacement, severe proteinuria). There is NO inflammatory reaction.

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9
Q

What can be seen on histology, immunofluorescence, and EM with membranous nephropathy?

A
  • histo: “spike and dome” on silver stain w/ GBM staining black (no inflamm. or prolif., but a thickened mem.)
  • immunofluor: granular deposits of IgG and C3
  • EM: subepithelial deposits (dark gray); thickening of mem. apparent
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10
Q

What is the prognosis of membranous nephropathy?

A

1/3 spontaneous remission, 1/3 develop renal failure and require dialysis, 1/3 continue to have proteinuria w/o progression to renal failure

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11
Q

What is the treatment for membranous nephropathy?

A

Treatment is difficult but often done with immunosuppressive drugs (like Prednisone). It is also important to treat the underlying cause if membranous nephropathy is secondary to another condition, like SLE.

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12
Q

In idiopathic cases of membranous nephropathy, what is the target antigen?

A

phospholipase A2 receptor (PLA2R); therefore, can do Ab testing for PLA2R as a diagnostic measure

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13
Q

Is there a decrease in complement with membranous nephropathy?

A

No, as this is a chronic and relatively slowly progressing disease. Thus, the liver has time to keep up with replenishing complement proteins.

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14
Q

Membranous nephropathy may be secondary to which non-autoimmune pathologies?

A

hyperlipidemia, hypercholesterolemia, accelerated atherogenesis

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15
Q

Which are the immune complex vs. non-immune complex mediated nephrotic syndromes?

A
  • immune complex: membranous nephropathy

- non-immune complex: Minimal Change Disease, Focal and Segmental Glomerulosclerosis (FSGS)

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16
Q

What is the classic clinical presentation of a patient with minimal change disease?

A

edema (periorbital or generalized)

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17
Q

What is the typical patient population affected by minimal change disease?

A

children 2-6 years old (only affects 10% of adults)

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18
Q

most common cause of nephrotic syndrome in children

A

minimal change disease

19
Q

What is the etiology of minimal change disease?

A
  • reversible podocyte injury
  • depression of immunity (viral infections, Hodgkin disease)
  • NSAIDs
  • maybe T-cell derived (cytokines)
20
Q

Minimal change disease involves [reversible/irreversible] podocyte injury.

A

reversible

21
Q

Is minimal change disease a relatively mild or severe disease?

A

Mild! Relapses of nephrotic syndrome are common, but it never progresses to kidney failure. It usually resolves at puberty.

22
Q

What is the treatment for minimal change disease?

A

steroids

23
Q

What is the patient population commonly affected by FSGS?

A
  • adults (35%) - most frequent diagnosis in human kidney biopsy
  • older children (10%)
  • African American and Hispanic patients
24
Q

What is the etiology/pathogenesis of FSGS?

A

irreversible** injury to podocytes

25
Q

What is the typical clinical presentation of a child with FSGS?

A
  • nephrotic syndrome
  • higher incidence of hematuria, reduced GFR, and HTN
  • non-selective proteinuria (more often)
26
Q

Why is there irreversible podocyte injury in FSGS, despite treatment?

A

This is because the disease primarily affects adults, and mature podocytes have limited capacity to replicate. Thus, irreversible podocyte injury leads to podocyte depletion with scarring (sclerosis).

27
Q

_________ has a common phenotype with diverse etiologies.

A

FSGS

28
Q

What are the 3 most common forms of FSGS (+ their causes)?

A
  • Primary FSGS: idiopathic, due to a circulating factor (? cytokine)
  • Adaptive FSGS: adaptive response to nephron loss (obesity, HTN, body-building, premature birth, sickle cell disease)
  • APOL1 FSGS: genetic risk alleles in APOL1 in AA’s (70% of idiopathic FSGS cases in AAs relate to APOL1)
29
Q

What are the 3 less common forms of FSGS?

A
  • Genetic FSGS
  • Medication/toxin-associated FSGS
  • Viral FSGS
30
Q

Genetic variants in the _____ gene account for a large fraction of the high rates of nondiabetic kidney disease in African Americans.

A

APOL1

31
Q

What is the prognosis of FSGS?

A

progression to renal failure (at least 50% end stage kidney disease within 10 years)

32
Q

What is the treatment of FSGS?

A
  • initially may be steroid-responsive (mimics minimal change disease)
  • progressively steroid-dependent/resistant
  • recurrence in kidney transplants
33
Q

Minimal change disease is more common in ______ (___%), whereas FSGS is more common in ______ (___%).

A

children, 65%; adults, 35%

34
Q

Which renal diseases are both nephrotic and nephritic (nephrotic syndrome + hematuria)?

A
  • Membranoproliferative Glomerulonephritis

- Dense Deposit Disease / C3 Glomerulonephropathy

35
Q

“double contour” or “tram track” appearance on silver stain

A

MPGN

36
Q

What is the etiology of MPGN?

A
  • primary: immune complex formation w/ classical complement activation
  • secondary: chronic autoimmune disorders, hepatitis, endocarditis, chronic bacterial infection
37
Q

What are the pathologic findings associated with MPGN?

A
  • lobular tufts
  • thick GBM w/ double contour
  • IgG + complement
38
Q

What is the best treatment of MPGN?

A

treatment of the underlying disease

39
Q

Which factors are involved with stabilizing vs. degrading C3 convertase?

A
  • C3NeF (C3 nephritic factor) stabilizes C3 convertase by binding to it and preventing its degradation
  • H factor degrades C3 convertase; so, mutations in gene encoding factor H, factor H deficiency, etc. may cause excess degradation of C3 convertase and therefore excess activation of the complement
40
Q

Dense Deposit Disease is a type of _______.

A

membranoproliferative glomerulonephritis (formerly MPGN type II)

41
Q

What is the main pathophysiology behind DDD?

A

excessive complement activation via alternative pathway, but NOT antibody-mediated

42
Q

What are the human diseases that involve excessive activation of the alternative complement pathway?

A
  • Glomerular: DDD / C3 glomerulonephritis

- Systemic: thrombotic microangiopathies

43
Q

What is seen on IF and EM with DDD?

A
  • IF: complement alone (C3) but NO immunoglobulin

- EM: dense deposits w/ lamina densa

44
Q

What is the treatment of DDD?

A

new therapies controlling complement activation (Eculizumab)

*MHD Exam 5 (14 decks)

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