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USMLE STEP 2 and 3 > Glomerular Diseases > Flashcards

Glomerular Diseases Flashcards

membranous nephropathy, Rapidly progressive GN

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USMLE® Step 1 flashcards
1
Q

What is the most common cause of primary membranous nephropathy?

A

Autoantibodies against the phospholipase A2 receptor (PLA2R) on podocytes.

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2
Q

What is the most common cause of nephrotic syndrome worldwide?

A

membranous nephropathy

Membranous GN is the most common cause for nephrotic syndrome in adults.

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3
Q

What is the role of anti-PLA2R antibody testing in membranous nephropathy?

A

It confirms the diagnosis of primary membranous nephropathy and can be used to monitor disease activity. Anti-PLA2R antibodies are highly specific for primary membranous nephropathy, confirming the diagnosis and helping distinguish it from secondary causes.

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4
Q

What are common secondary causes of membranous nephropathy?

A

Infections: Hepatitis B, Hepatitis C, Syphilis

Autoimmune conditions: Systemic lupus erythematosus (class V lupus nephritis), thyroiditis

Drugs: NSAIDs, penicillamine, gold

Malignancies: Solid tumors (lung, breast, colon cancer)

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5
Q

What should be investigated if a biopsy specimen is negative for anti-PLA2R antibodies in membranous nephropathy?

A

Secondary causes such as malignancy, infection (e.g., hepatitis B or C), autoimmune disease, or drug-related nephropathy should be evaluated.

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6
Q

What malignancies are commonly associated with secondary membranous nephropathy in elderly patients?

A

Solid tumors such as lung, breast, and colon cancer are commonly linked to secondary membranous nephropathy.

Should preform age-appropriate cancer screens for patients with idiopathic nephropathy.

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7
Q

If anti-PLA2R antibodies are absent, what is the next diagnostic step in elderly patients with membranous nephropathy?

A

Perform cancer screening, such as colonoscopy, mammography, or CT imaging, to identify secondary causes.

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8
Q

What type of cancer can cause nephropathy but not membranous nephropathy?

A

Multiple myeloma can cause amyloidosis leading to amyloid nephropathy

Congo red staining, which demonstrates apple-green birefringence under polarized light.

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9
Q

What is the characteristic histological finding in membranous nephropathy on light microscope?

A

Diffuse capillary and GBM thickening

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10
Q

What is the characteristic histological finding in membranous nephropathy on immunofluorescence?

A

granular deposition

Membranous nephropathy is an immunologically mediated disease in which deposts of mainly IgG and complement collect in the basement membrane and appear in a diffuse granular pattern

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11
Q

What is the characteristic histological finding in membranous nephropathy on electron microscope?

A

Subepithelial immune complex deposits seen on electron microscopy, with spike and dome appearance on silver stain.

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12
Q

What special stain is useful in evaluating membranous nephropathy?

A

Silver stain

There are characteristic “spikes” of basement membrane between the immune deposits of membranous nephropathy. The black basement membrane material shown here appears as projections in the capillary loops.

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13
Q

What is the clinical presentation of membranous nephropathy?

A

Nephrotic syndrome, including proteinuria >3.5 g/day, hypoalbuminemia, edema, and hyperlipidemia.

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14
Q

Why is testing for C3 complement protein unnecessary in membranous nephropathy?

A

C3 complement protein testing is relevant for diseases like postinfectious glomerulonephritis or C3 glomerulopathy, which involve low C3 levels. Membranous nephropathy does not typically involve hypocomplementemia.

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15
Q

How is IgA nephropathy differentiated from membranous nephropathy?

A

IgA nephropathy presents with glomerulonephritis, not nephrotic syndrome, and is associated with anti-IgA antibodies, not anti-PLA2R antibodies.

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16
Q

How can Goodpasture’s syndrome be differentiated from membranous nephropathy?

A

Goodpasture’s syndrome is associated with anti-glomerular basement membrane (anti-GBM) antibodies, presenting with nephritic syndrome and often pulmonary hemorrhage.

17
Q

What laboratory findings are common in membranous nephropathy?

A

Urinalysis: Proteinuria, lipiduria, microscopic hematuria

Serum studies: Hypoalbuminemia, hypercholesterolemia, elevated serum creatinine in advanced disease.

18
Q

How is secondary membranous nephropathy diagnosed?

A

By identifying the underlying cause through specific tests such as Hepatitis B/C serologies, ANA testing, or cancer screening.

19
Q

What is the first-line treatment for primary membranous nephropathy?

A

Supportive therapy with RAAS blockade (ACE inhibitors/ARBs) for proteinuria and blood pressure control.

Immunosuppressive therapy is used for high-risk cases (e.g., nephrotic-range proteinuria or kidney dysfunction) when the cause is idiopathic.

20
Q

What immunosuppressive agents are used in membranous nephropathy?

A

Corticosteroids combined with cyclophosphamide or calcineurin inhibitors
(e.g., tacrolimus or cyclosporine).

21
Q

What complications are associated with membranous nephropathy?

A

Thromboembolism (due to hypercoagulability in nephrotic syndrome).

Chronic kidney disease with partial remission common.

Rarely there is a progression to end-stage renal disease (ESRD).

22
Q

What is Rapidly Progressive Glomerulonephritis (RPGN)?

A

A syndrome of rapid renal function loss over days to weeks, characterized by crescentic glomerulonephritis on biopsy seen on either light microscope or immunofluorescence.

23
Q

What is the hallmark pathology of RPGN on biopsy?

A

Crescent moon shapes of fibrin on light microscopy (LM).

24
Q

Clinical Presentation of RPGN

A

Rapidly declining renal function.
Oliguria or anuria.
Systemic symptoms (e.g., hemoptysis in Goodpasture, purpura in ANCA vasculitis).

Labs:
Elevated serum creatinine and BUN.
Dysmorphic red blood cells or red blood cell casts in urine.
Proteinuria.

25
Q

What are the three types of RPGN?

A

Type I (Anti-GBM):
Goodpasture syndrome.

Type II (Immune Complex):
Lupus nephritis, post-streptococcal GN.

Type III (Pauci-immune):
Granulomatosis with polyangiitis, microscopic polyangiitis, Churg-Strauss syndrome.

26
Q

What diagnostic patterns are seen in RPGN on immunofluorescence (IF)?

A

Type I (Anti-GBM): Linear pattern.
Type II (Immune Complex): Granular pattern.
Type III (Pauci-immune): No immunofluorescence (pauci-immune).

27
Q

What is Goodpasture syndrome and its key features?

A

Autoimmune disease with anti-GBM antibodies causing hemoptysis (pulmonary hemorrhage) and glomerulonephritis.

28
Q

Type I RPGN

A

Etiology: Autoantibodies against glomerular basement membrane (GBM).

Clinical Features: Hemoptysis (pulmonary hemorrhage) and glomerulonephritis.

Diagnosis: Linear IF with anti-GBM antibodies.

Treatment: High-dose corticosteroids, cyclophosphamide, and plasmapheresis.

29
Q

How is Goodpasture syndrome diagnosed?

A

Linear immunofluorescence pattern with anti-GBM antibodies.

30
Q

What is the treatment for Goodpasture syndrome?

A

High-dose corticosteroids, cyclophosphamide, and plasmapheresis.

31
Q

Type II RPGN

A

Etiology: Immune complex deposition from systemic diseases (e.g., lupus nephritis, post-streptococcal GN).

Clinical Features: Signs of underlying systemic disease.

Diagnosis: Granular IF pattern with immune complex deposits.

Treatment: High-dose corticosteroids and disease-specific therapy.

32
Q

What causes Type II RPGN?

A

Immune complex deposition from systemic diseases like lupus nephritis or post-streptococcal GN.

33
Q

What is seen on IF in Type II RPGN?

A

Granular pattern due to immune complex deposits.

34
Q

What is the treatment for Type II RPGN?

A

High-dose corticosteroids and therapy specific to the underlying disease.

35
Q

Type III RPGN

A

Etiology: Associated with ANCA-associated vasculitides (e.g., granulomatosis with polyangiitis).

Clinical Features: Systemic vasculitis symptoms (e.g., hemoptysis, sinusitis, purpura).

Diagnosis: No IF staining; ANCA positive.
c-ANCA: Granulomatosis with polyangiitis.
p-ANCA: Microscopic polyangiitis or Churg-Strauss syndrome.

Treatment: Corticosteroids, cyclophosphamide, and rituximab.

36
Q

What diseases are associated with Type III RPGN?

A

ANCA-associated vasculitides:

Granulomatosis with polyangiitis
Microscopic polyangiitis
Churg-Strauss syndrome

37
Q

What diagnostic findings are seen in Type III RPGN?

A

No immunofluorescence (pauci-immune)

and

ANCA positivity
(c-ANCA for granulomatosis with polyangiitis or p-ANCA for microscopic polyangiitis).

38
Q

How is Type III RPGN treated?

A

Corticosteroids, cyclophosphamide, and rituximab.

39
Q

What is the general treatment for RPGN?

A

High-dose corticosteroids, cyclophosphamide, and plasmapheresis (if anti-GBM antibodies are present).

USMLE STEP 2 and 3 (50 decks)

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