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STEP 7: Renal Urinary Tract PATHOMA FA CORBETT > NephrOtic/Nephritic Syndromes PATHOMA/FA > Flashcards

NephrOtic/Nephritic Syndromes PATHOMA/FA Flashcards

1
Q

What is the main characteristic of NEPHROTIC glomerular disorders?

A

PROTEINURIA - Protein in urine >3.5g/day

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2
Q

Name the 3 proteins that are particularly LOW in NEPHROTIC SYNDROMES and the results.

A
  1. HYPOALBUMINEMIA -> Decreased intravascular oncotic pressure -> PITTING EDEMA
  2. HYPOGAMMAGLOBULINEMIA -> Increased risk of INFECTION
  3. Decreased ANTITHROMBIN III -> HYPERCOAGULABLE STATE
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3
Q

What molecules are particularly elevated in pts with NEPHROTIC SYNDROMES? As a result, what can be seen in the urine?

A

HYPERLIPIDEMIA + HYPERCHOLESTEROLEMIA - ‘Blood loses lots of protein, liver responds by throwing out fats to try to beef up the blood’
RESULT: FATTY CASTS in urine

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4
Q

What is the most common cause of NEPHROTIC SYNDROME in children?

A

MINIMAL CHANGE DISEASE

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5
Q

What syndrome/disease is MINIMAL CHANGE DISEASE (MCD) particularly associated with?

A

HODGKIN LYMPHOMA

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6
Q

What layer of the glomerular filtration barrier is effaced (Flattened) in MCD?

A

FOOT PROCESSES OF PODOCYTES (i.e. EPITHELIAL CELLS)

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7
Q

IMAGING of MINIMAL CHANGE DISEASE: What do you see on H&E, EM, and IF?

A

H&E: Normal glomerulus
EM: Flattened (effaced) foot processes
IF: NEGATIVE (no immune complex deposits)

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8
Q

Which NEPHROTIC SYNDROME is the only one that responds EXCELLENTLY to steroids? Why?

A

MCD
Because effaced podocytes in MCD result from a high [cytokine] whether it’s idiopathic or from Hodgkin’s lymphoma
Steroids = Less cytokine production

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9
Q

Which proteins are lost in MINIMAL CHANGE DISEASE?

A

ONLY ALBUMIN

‘Think of it as MCD is so minimal that only albumin is lost’

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9
Q

Are NEPHROTIC syndromes generally familial/genetic, environmental, or idiopathic? Do they generally respond well to STEROID therapy?

A

IDIOPATHIC - most common

Do NOT respond well to steroids - except for MCD

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10
Q

Which is the most common cause of NEPHROTIC SYNDROME in Hispanics and African Americans?

A

FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS)

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11
Q

Which pt populations are most likely to have FSGS, if they have a NEPHROTIC SYNDROME?

A
  1. HIV
  2. Heroine usage
  3. Sickle cell disease
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12
Q

What is the imaging seen on FSGS? Include H&E, EM, and IF

A

H&E: Segmental + focal Sclerosis (dense pink collagen deposits) in glomeruli
EM and IF - same as MCD bec it is almost a progression of MCD that was unresponsive to steroids [Effaced foot processes, negative IF]

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13
Q

What is the most common cause of NEPHROTIC SYNDROME in Caucasian adults?

A

MEMBRANOUS NEPHROPATHY

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14
Q

What is the most common cause of death in SLE pts? What is the most common specific disease responsible for it?

A

RENAL FAILURE - Specifically Diffuse Proliferative Glomerulonephritis

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15
Q

If a SLE pt had proteinuria >3.5g/day, pitting edema (hypoalbuminemia), increased risk of infection (hypogammaglobulinemia), hypercholesterolemia, and clots very easily, what particular disease is this pt most likely suffering through?

A

Pt has NEPHROTIC SYNDROME - most common renal nephrotic disease in SLE pt is MEMBRANOUS NEPHROPATHY

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16
Q

What are the non-idiopathic associations of MEMBRANOUS NEPHROPATHY?

A

HEPATITIS B/C + Solid tumors + SLE + Drugs (NSAIDS, PENICILLINAMINE)

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17
Q

What is the ddx (renal pathology) when Immunofluorescence is GRANULAR?

A

GRANULAR IF = IMMUNE COMPLEX DEPOSITS
Ddx: NEPHROTIC (2): Membranous Nephropathy + Membranoproliferative Glomerulonephritis
NEPHRITIC (all)

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18
Q

What two disorders have thickened glomerular membranes due to immune complex depositions?

A

Renal disorders that have “membranous” - MEMBRANOUS NEPHROPATHY + MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS

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19
Q

SPIKE and DOME appearance on EM: What renal disease am I?

A

MEMBRANOUS NEPHROPATHY

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20
Q

What is shown on imaging of MEMBRANOUS NEPHROPATHY? List H&E, EM, and IF.

A

H&E: Thickened glomerular membranes
EM: Spike and dome - subepthelial deposits
IF: Granular IF - immune complex

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21
Q

Which nephrotic syndrome is MOST commonly associated with TRAM TRACK appearance on H&E stain?

A

TYPE I (SUBENDOTHELIAL) MPGN

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22
Q

What is the imaging results of MPGN? List HE, EM, and IF.

A

H&E and EM: Thickened capillary membrane with TRAM TRACK (more common with Type I MPGN)
IF: Granular due to immune complex deposits

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23
Q

What is the proliferative component of MPGN?

A

MESANGIAL CELLS proliferate their cytoplasm -> Cuts into the immune complex deposit -> TRAM TRACKS

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24
Q

What diseases is TYPE I MPGN most associated with?

A

HBV and HCV

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25
Q

What condition is TYPE 2 MPGN most associated with?

A

Associated with C3 NEPHRITIC FACTOR - which stabilizes C3 CONVERTASE -> LOW C3 + HIGH C3a/C3b -> Complement overactivation -> Glomerular damage

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26
Q

What is unique of MPGN in terms of classification: NephrOtic, NephrItic?

A

MPGN can be Nephrotic, Nephritic (moreso Type II MPGN - inflammation related), or BOTH

27
Q

What are the two causes of HYALINE ARTERIOSCLEROSIS?

A
  1. BENIGN HTN

2. DIABETES MELLITUS: Nonenzymatic glycosylation of vascular BM -> Protein leakage into BV wall -> HA

28
Q

What is the first change to the kidney seen in DIABETES MELLITUS?

A

Non-enzymatic glycosylation of the vascular membrane -> HYALINE ARTERIOSCLEROSIS

29
Q

Where does DM-mediated HYALINE ARTERIOSCLEROSIS preferentially occur?

A

EFFERENT ARTERIOLE

30
Q

What happens as a result of EFFERENT ARTERIOLE HYALINE ARTERIOSCLEROSIS seen in a diabetic kidney?

A

INCREASE in GFR -> Hyperfiltration-induced damage -> MICROALBUMINURIA

31
Q

In late-stage DM, what renal syndrome is developed in the kidney? What is the pathogenesis?

A

NEPHROTIC SYNDROME develops after mesangial sclerosis

32
Q

KIMMELSTIEL-WILSON NODULES are pathognomonic of __? What are these nodules?

A

DIABETES MELLITUS

Nodules that contain significant MESANGIAL SCLEROSIS

33
Q

What treatment can slow the progression of Hyperfiltration-induced glomerular damage of a pt with DIABETES MELLITUS? How?

A

ACE INHIBITORS - Block AngII’s efferent arteriolar vasoconstriction that would also INCREASE GFR and cause hyperfiltration

34
Q

What is the most commonly involved organ in SYSTEMIC AMYLOIDOSIS? How specifically does amyloidosis affect this organ?

A

KIDNEY - Amyloid deposits in the MESANGIUM -> Nephrotic syndrome

35
Q

What is the confirmatory test of SYSTEMIC AMYLOIDOSIS?

A

CONGO RED STAIN: Apple-green birefringence under polarized light

36
Q

What is the most common cause of END-STAGE RENAL DISEASE in the US?

A

DIABETIC GLOMERULONEPHROPATHY

37
Q

What are characteristics of the urine in NEPHRITIC SYNDROME?

A
  1. LIMITED proteinuria (
38
Q

What are the most prototypical Sx of NEPHRITIC SYNDROME?

A

PERIORBITAL EDEMA + HTN - due to salt and secondary water retention
Minimal edema presents in the loose CT around the eyes

39
Q

What does a biopsy of NEPHRITIC SYNDROME glomerulus look like?

A

HYPERCELLULAR, INFLAMED glomeruli due to:

Immune complex deposition -> Activates complement -> C5a attracts neutrophils (hypercellular)

40
Q

SUBEPITHELIAL HUMPS on EM = ____?

A

POST-STREP GLOMERULONEPHRITIS (PSGN)

41
Q

What are the imaging results of PSGN. List HE, EM, IF.

A

HE: Hypercellular, inflamed glomeruli
EM: SUBEPITHELIAL DEPOSITS (Immune complex deposits that originated SUBENDOTHELIAL travel up above BM)
IF: Granular

42
Q

What infection elicits PSGN?

A
  1. GROUP A beta-hemolytic STREPTOCCOCAL infection of the pharynx or skin (impetigo) - nephritogenic strain containing M protein (virulence factor)
  2. NON-STREPTOCOCCAL infection
43
Q

What is the treatment of PSGN? What is the basis for this treatment?

A

SUPPORTIVE - Only supportive because the SUBEPITHELIAL complex deposits eventually dissipate out and disappear

44
Q

What is the most common age population of PSGN?

A

Children

45
Q

What is the difference in terms of potential complications of PSGN in CHILDREN and ADULTS?

A

More common pt pool: Children - Only 1% can progress to RENAL FAILURE
Adults - 25% can progress to RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS

46
Q

What is the distinguishing clinical sign when comparing RPGN of GOODPASTURE vs. WEGENER GRANULOMATOSIS? What is the distinguishing IF imaging pattern?

A

GOODPASTURE: IF is LINEAR (auto-Ab against collagen on alveolar and glomerular membranes) + NO NASOPHARYNX involvement
WEGENER: IF is NEGATIVE, so ANCA test is done and shows c-ANCA + YES NASOPHARYNX involvement (sinusitis/history of sinus infections)

47
Q

What is seen on HE imaging of RPGN? What is this particular feature made up of?

A

CRESCENT in Bowman’s space = Inflammatory debris = FIBRIN + MACROPHAGES

48
Q

Pt with RPGN has an IF result of SHARP, LINEAR. What disease does pt have?

A

GOODPASTURE - Auto-Ab against collagen in alveolar and glomerular BM

49
Q

Pt with RPGN has a NEGATIVE IF. What is on my Ddx now? What test does pt now need to get?

A

Ddx: Wegener granulomatosis, Microscopic Polyangiitis, and Churg-Strauss Sydnrome
Pt needs to get ANCA test

50
Q

What is an ANCA test? What are the two possible results? What RPGN disease are associated with each?

A

Anti-neutrophil cytoplasmic Antibody test

  1. c-ANCA (cytoplasmic) = Wegener Granulomatosis
  2. p-ANCA (perinuclear) = Microscopic Polyangiitis + Churg Strauss Syndrome
51
Q

How do you distinguish CHURG-STRAUSS SYNDROME from MICROSCOPIC POLYANGIITIS as two probable causes of RPGN with a p-ANCA IF result?

A

CHURG-STRAUSS has 3 features that are ABSENT in microscopic polyangiitis: GRANULOMATOUS INFLAMMATION + EOSINOPHILIA + ASTHMA

52
Q

Pt has RBC casts in the urine, oliguria, proteinuria

A

Pt has NEPHRITIC Syndrome, not Nephrotic

Ddx: PSGN + DPGN (diffuse proliferative glomerulonephritis)

53
Q

In a pt with NEPHRITIC SYNDROME and GRANULAR IF, what is the most common/probable disease that the pt has?

A

PSGN - POST STREPTOCOCCAL GLOMERULONEPHRITIS

54
Q

In a SLE pt with NEPHRITIC SYNDROME and GRANULAR IF, what is the most common/probable disease that the pt has?

A

DPGN - DIFFUSE PROLIFERATIVE GLOMERULONEPHRITIS

55
Q

Which is the one renal disease with IgA complex deposition?

A

BERGER DISEASE - IgA NEPHROPATHY

56
Q

What is the most common nephropathy worldwide?

A

IgA NEPHROPATHY (BERGER DISEASE)

57
Q

What is the most common cause of BERGER DISEASE? Explains the pathogenesis.

A

MUCOSAL INFECTIONS -> Excessive IgA production -> IgA deposits in MESANGIUM of glomeruli

58
Q

Why is the HEMATURIA seen in BERGER DISEASE (IgA NEPHROPATHY) episodic?

A

Bec Berger Disease is elicited by IgA complexes, and you get IgA with episodic mucosal infections. When infection subsides, IgA disappears

59
Q

ALPORT SYNDROME is a defect in what molecule? Is ALPORT sporadic or inherited? If inherited, what is the inheritance pattern?

A

Defect in TYPE 4 COLLAGEN

INHERITED - X-linked

60
Q

What are the 3 clinical Sx of ALPORT SYNDROME based on the thinning and splitting of basement membranes?

A

SENSORY HEARING LOSS + OCULAR DISTURBANCES + ISOLATED HEMATURIA (BM of glomeruli and ocular system)

61
Q

What are the 4 NEPHRITIC SYNDROMES of the kidney?

A

‘2 + 2 names’

  1. POST-STREPTOCOCCAL GLOMERULONEPHRITIS (PSGN)
  2. RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS (RPGN)
  3. BERGER DISEASE (IgA nephropathy)
  4. ALPORT SYNDROME
62
Q

What type of immune reaction is exhibited in ACUTE PSGN? (post-streptococcal glomerulonephritis)

A

TYPE III HSR

63
Q

What type of immune complexes are seen in PSGN and where are they located?

A

IgG, IgM, and C3 deposits along MESANGIUM and GBM

64
Q

What is the most common AutoAg of which Abs are produced against in MEMBRANOUS GLOMERULONEPHROPATHY?

A

Phospholipase A2 Receptor

STEP 7: Renal Urinary Tract PATHOMA FA CORBETT (7 decks)

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