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Flashcards in Rheum 2 Deck (30):
1

What is the number 1 characterization of Seronegative Spondyloarthropathies

sacroiliitis (SI) joint stiffness - axial arthritis

2

Enthesitis
- what is it?
- what rheum disorder is it seen in?

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

- seen in Seronegative Spondyloarthropathies

3

genetic association of Seronegative Spondyloarthropathies?
- RF?
- ANA?

HLA-B27

neg RF and ANA

4

Synovium of Seronegative Spondyloarthropathies

increased expression of TNFα

5

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

2%

20%

6

Reactive arthritis, presentation?

Asymmetric, oligoarticular, lower ext arthritis!!
Dactylitis

7

Reiter’s syndrome associated with what disorder?

Reactive arthritis

8

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

Sausage digits - reactive arthritis

9

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

Reactive arthritis

10

Fundamental defect of SLE?

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

11

Which lymphocytes are involved in the autoimmune process of SLE?

T and B cell

12

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

antigen
CD4+ T cells

13

In SLE, Loss of T cell tolerance permits what?

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

14

greatest risk factor of SLE?
what other genetic factor is associated with SLE?

C4A null allele

HLA-DR3, +ANA

15

What are ANAs?

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

16

What hypersensitivity is SLE?

II and III (lumpy bumpy)

17

Anti-phospholipid antibodies in SLE are associated with what?

increased clotting

18

Types of cells in Vasculitis?

infiltrating lymphs, monocytes, histiocytes, eosinophils, and PMNs

19

Pathophysiology of vasculitis

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

- antigen driven inflammation

20

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

Cytoplasmic ANCA (c-ANCA):

Proteinase-3 (PR3) in primary granules of PMNs

- Associated with generalized GPA (Wegener’s)

21

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

Perinuclear ANCA (p-ANCA):

Myeloperoxidase (MPO) in primary granules of PMNs

Associated with microscopic polyangiitis (MPA)

22

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

Likely play a role in amplifying the inflammatory vascular response

23

Polymyositis / Dermatomyositis (PM/DM)

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

24

DM presents with typical skin rashes:

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

25

Anti-synthetase Syndrome

PM or DM presenting with:
Interstitial lung disease (ILD): 60%

26

Anti-synthetase antibodies:
- list the enzyme class, then the most common one

Anti-aminoaCYl-tRNA synthetases (CYtoplasm)

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

27

Are the antibodies in Anti-synthetase Syndrome pathologic?

Not pathologic or myotoxic antibodies


*recall, RF-IgG immune complexes ARE pathogenic

28

Polymyositis
- what type of distribution?
- Distribution in which T cells?

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

29

Dermatomyositis
- what type of distribution?
- Distribution in which T cells?

Perivascular (CD4+ T cells) and perifascicular inflammatory infiltrate

30

What rheum disorder is associated with a viral etiology?
Has live virus been cultured?

PM/DM

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