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BL DD final > Rheum 2 > Flashcards

Rheum 2 Flashcards

1
Q

What is the number 1 characterization of Seronegative Spondyloarthropathies

A

sacroiliitis (SI) joint stiffness - axial arthritis

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2
Q

Enthesitis

  • what is it?
  • what rheum disorder is it seen in?
A

inflammation of the ligamentous-, tendinous-, fibrous-osseous junctions (where they insert into bone)

  • seen in Seronegative Spondyloarthropathies
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3
Q

genetic association of Seronegative Spondyloarthropathies?

  • RF?
  • ANA?
A

HLA-B27

neg RF and ANA

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4
Q

Synovium of Seronegative Spondyloarthropathies

A

increased expression of TNFα

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5
Q

For Seronegative Spondyloarthropathies (ankylosing spondylitis AS)
Chances of developing AS is…
_____ % if HLA-B27 positive
_____% if HLA-B27 positive with a first-degree relative with AS

A

2%

20%

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6
Q

Reactive arthritis, presentation?

A

Asymmetric, oligoarticular, lower ext arthritis!!

Dactylitis

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7
Q

Reiter’s syndrome associated with what disorder?

A

Reactive arthritis

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8
Q

Dactylitis, what is it? what is it associated with?

A

Sausage digits - reactive arthritis

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9
Q

HLA-B27 Unfolded Protein Hypothesis Endoplasmic Reticulum Stress Response
is associated with what disorder?

A

Reactive arthritis

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10
Q

Fundamental defect of SLE?

A

misdirected recognition of self as foreign, resulting in an autoimmune process; T cell and B cell process

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11
Q

Which lymphocytes are involved in the autoimmune process of SLE?

A

T and B cell

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12
Q

In SLE, Antibody responses toward autoantigens are _____-driven and require ______

A

antigen

CD4+ T cells

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13
Q

In SLE, Loss of T cell tolerance permits what?

A

B cell stimulation: peripheral abnormality in self-reactive lymphocyte deletion or anergy (state of unresponsiveness)

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14
Q

greatest risk factor of SLE?

what other genetic factor is associated with SLE?

A

C4A null allele

HLA-DR3, +ANA

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15
Q

What are ANAs?

A

Hallmark of abnormal antibody production in SLE (not specific), Antibodies are directed to multiple nuclear antigens

  • dont mix it up, C4A null allele still the most imp risk factor of SLE
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16
Q

What hypersensitivity is SLE?

A

II and III (lumpy bumpy)

17
Q

Anti-phospholipid antibodies in SLE are associated with what?

A

increased clotting

18
Q

Types of cells in Vasculitis?

A

infiltrating lymphs, monocytes, histiocytes, eosinophils, and PMNs

19
Q

Pathophysiology of vasculitis

A

Immune complexes: inflamm→ PAFs→ ↑vascular permeability→ IC deposition; palpable purpura

  • antigen driven inflammation
20
Q

ANCA Vasculitis:
c-ANCA
- what are located in primary granules of PMNs, and what disorder is it associated with?

A

Cytoplasmic ANCA (c-ANCA):

Proteinase-3 (PR3) in primary granules of PMNs

  • Associated with generalized GPA (Wegener’s)
21
Q

ANCA Vasculitis:
p-ANCA
- what are located in primary granules of PMNs, and what disorder is it associated with?

A

Perinuclear ANCA (p-ANCA):

Myeloperoxidase (MPO) in primary granules of PMNs

Associated with microscopic polyangiitis (MPA)

22
Q

Antineutrophil Cytoplasmic Antibodies (ANCAs): play a role in what?

A

Likely play a role in amplifying the inflammatory vascular response

23
Q

Polymyositis / Dermatomyositis (PM/DM)

A 
Inflammatory myopathies characterized by:
Muscle weakness (proximal) and low endurance
24
Q

DM presents with typical skin rashes:

A 
Gottron’s papules 
Heliotrope rash 
V-sign and shawl-sign 
Mechanic’s hands 
Periungual changes/erythema
25
Q

Anti-synthetase Syndrome

A

PM or DM presenting with:

Interstitial lung disease (ILD): 60%

26
Q

Anti-synthetase antibodies:

- list the enzyme class, then the most common one

A

Anti-aminoaCYl-tRNA synthetases (CYtoplasm)

Most common: Anti-Jo-1 = anti-histadyl-tRNA synthetase

27
Q

Are the antibodies in Anti-synthetase Syndrome pathologic?

A

Not pathologic or myotoxic antibodies

*recall, RF-IgG immune complexes ARE pathogenic

28
Q

Polymyositis

  • what type of distribution?
  • Distribution in which T cells?
A

Endomysial distribution of inflammatory cells (CD8+ T cells) surrounding and invading muscle fibers

29
Q

Dermatomyositis

  • what type of distribution?
  • Distribution in which T cells?
A

Perivascular (CD4+ T cells) and perifascicular inflammatory infiltrate

30
Q

What rheum disorder is associated with a viral etiology?

Has live virus been cultured?

A

PM/DM

Viral particles by EM and viral RNA detected in muscle from PM/DM patients. NO live virus has been cultured from muscle

BL DD final (18 decks)

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