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Ch 32 Secondary GN > FIBRILLARY GN & IMMUNOTACTOID GN > Flashcards

FIBRILLARY GN & IMMUNOTACTOID GN Flashcards

1
Q

What is the size of fibrils in Fibrillary GN?

A

16-24 nm (mean: 20nm)

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2
Q

What is the size of fibrils in Immunotactoid GN?

A

• 30-50 nm
• hollow microtubules

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3
Q

What is a a specific immunohistochemical marker found in the distribution of the fibrillary glomerular deposits?

A

DNAJB9

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4
Q

What is a rare form of fibrillary renal disease in which the glomeruli are infiltrated by massive deposits of fibronectin?

A

FIBRONECTIN GLOMERULOPATHY

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5
Q

What type of GN occurs mostly in adults, in both sexes & in all age groups?

A

FIBRILLARY GN

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6
Q

What are the 3 disorders associated with Fibrillary GN?

A
  1. MALIGNANCY
  2. MONOCLONAL GAMMOPATHY
  3. AUTOIMMUNE DISORDERS
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7
Q

Patients with this type of fibrillary GN tend to be older, may have a less rapidly progressive course

A

IMMUNOTACTOID GN

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8
Q

What disorder is associated with Immunotactoid GN?

A

LYMPHOPROLIFERATIVE DISORDERS

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9
Q

What infection is associated with Fibrillary & Immunotactoid GN?

A

HCV

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10
Q

What are the pathognomonic EM findings in Amyloid, Fibrillary & Immunotactoid GN?

A

AMYLOID: 8-12 nm
FIBRILLARY: 16-24 nm, non-branching
IMMUNOTACTOID: 20-30 nm, hollow

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11
Q

What type of GN where fibrils are arranged randomly in the mesangial matrix and GBMs?

A

FIBRILLARY GN

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12
Q

What type of GN where the microtubules are often arranged in parallel stacks in the mesangium, subendothelial, and/or subepithelial regions?

A

IMMUNOTACTOID GN

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13
Q

What is the IF staining in Fibrillary GN?

A

• POLYCLONAL
• always for IgG (IgG1 and IgG4)
• also C3, and both κ and λ chains,

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14
Q

What is the 2 most common IgG subclass in Fibrillary GN?

A

• IgG1
• IgG4

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15
Q

What is the IF staining in immunotactoid?

A

• MONOCLONAL
• IgG

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16
Q

What are the 2 most common IgG subclass associated with Immunotactoid GN?

A

• IgG1
• IgG2

17
Q

In both Fibrillary and Immunotactoid GN, deposits are limited to where?

A

GLOMERULUS

18
Q

What are the 2 treatments used for fibrillary GN with crescentic pattern on LM?

A
  1. CORTICOSTEROIDS
  2. CYCLOPHOSPHAMIDE
19
Q

What is the treatment used for fibrillary GN with membranous pattern on LM?

A

CYCLOSPORINE

20
Q

What is the treatment used for fibrillary GN with MPGN pattern on LM?

A

RITUXIMAB

21
Q

What is the type of Fibrillary Renal Disease which is a familial disease with autosomal dominant inheritance that presents with proteinuria and hematuria usually in adolescents and eventually progresses to nephrotic syndrome and slowly deteriorating renal function?

A

Fibronectin glomerulopathy

22
Q

Fibronectin glomerulopathy is caused by an inherited mutation in the gene encoding?

A

FIBRONECTIN-1

Ch 32 Secondary GN (35 decks)

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