Other Autoimmune (RA and Scleroderma) lecture Flashcards Preview

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Flashcards in Other Autoimmune (RA and Scleroderma) lecture Deck (19):
1

RA is a/w HLA-___.

DRB1

2

What cell type is primarily responsible for joint destruction in RA?

T-cell: There is B-cell too, but they are activated by T-cells

3

What is meant by pannus invasion of joints?

Synovial proliferation destroys adjacent bone and cartilage

4

What direction is typical deviation of wrists and fingers in RA?

Wrist - radial
Fingers - ulnar

5

What are the immunoglobulins most commonly involved in RA and what are their actions?

IgM against Fc portion of IgG

6

What type of hypersensitivity is responsible for the joint involvement in RA?

Type III - Immune complexes in synovial fluid and membrane

7

What titer is a/w the prognosis of RA?

RF+ is poor prognosis
RF- is good prognosis

8

40 y.o. female presents with proximal joint swelling and pain. She is also found to have rheumatoid factor titers and firm, moveable nodules on her fingers. What is expected on histo of the nodules?

Central fibrinoid necrosis surrounded by epithelial histiocytes, lymphs, and plasma cells.
Note: There is no RBC involvement (as opposed to gonococcal arthritis)

9

What cells are responsible for protease and elastase involvement in RA? What about collagenase, osteoclast activation, and endothelial adhesion?

Neutrophils and synoviocytes release protease and elastase
Macrophages and synoviocytes release IL-1 and TNF-a

10

What is the pathophysiology behind organ involvement in RA?

Vasculitis in small to medium arteries causes reduced blood flow and ischemia

11

Anti-topoisomerase is primarily a/w ______ _______.

Diffuse scleroderma

12

What is the antibody a/w CREST?

anti-centromere

13

What does CREST stand for?

Calcinosis
Raynaud's
Esophageal dysfunction
Sclerodactyly
Telangietesia: Dilated capillaries --> red/purple clusters

14

What two cell types are primarily responsible for the early phase of scleroderma?

CD4 and macrophages

15

What are the hallmark facial features of scleroderma?

Absence of wrinkles, skin tightness, and down turned or open mouth

16

What is the pathophysiology of ischemia in scleroderma (a.k.a. systemic sclerosis)?

Fibrotic intimal thickening and stenosis of small arteries leads to ischemia and end organ (and finger) death.

17

What is the first line treatment of renal crisis in scleroderma?

ACE inhibitors (-pril)

18

What is the leading cause of death in scleroderma?

Pulmonary fibrosis leading to for pulmonale

19

What are the two systems most involved in scleroderma?

Integumentary and GI