Systemic rheum disease

Question 1

4 features of collagen vascular diseases

Answer 1

1 inflammation - causes disease activity
2 immune activation - leads to loss of tolerance
3 vasculopathy - leads to tissue ischemia and necrosis
4 fibrosis - end stage from chronic inflammation - organ dysfunction

Question 2

how do systemic diseases present

Answer 2

generally undifferentiated and then become more classic over time

Question 3

mech. of SLE

Answer 3

loss of tolerance>auto-antibodies>

1. cytotoxic ABs>tissue inflammation and damage
2. immune complex formation>low complement and inflammation/damage

Question 4

3 ways to help diagnose SLE

Answer 4

1. bloods - cytopenia, renal involvemnet
2. ANAs
3. possibly kidney biopsy

Question 5

Sx in SLE

Answer 5

• rash
• scalp/alopecia
• glomerulonephritis (nephrotic syndrome)

Question 6

what is seen on serology in SLE (5)

Answer 6

• cytopenia
• ANA
• dsDNA
• anti smith
• low c3/c4

Question 7

2 key features of SLE

Answer 7

1. immune activation

2. inflammation

Question 8

prominent features of scleroderma

Answer 8

• NOT inflammation
• vasculopathy dominant
• thickening and fibrosis predominant

Question 9

4 common manifestations of vasculopathy

Answer 9

1. raynauds
2. fingertip ulcers
3. nailbed hemmorages
4. telangiectasias

Question 10

3 consequences of fibrotic changes in scleroderma

Answer 10

1. sclerodactyly
2. facial changes
3. interstitial lung disease

Question 11

CREST form of sclerodrma

Answer 11

Calcinosis
Raynauds
Eso dysmotility
Scerodactyly
Telantectasia

Question 12

typical presentation of inflammatory myositis

Answer 12

• PAINLESS proximal muscle weakness

Question 13

2 types

Answer 13

polymyositis - primarily in muscles

dermatomyositis - in skin too

Question 14

2 tests to help diagnose myositis

Answer 14

1. CK in blood

2. muscle biopsy

Question 15

2 features at work in mysotis

Answer 15

1. immune inactivation

2. inflammation

Question 16

3 other collagen vasc. disorders

Answer 16

1. sjorgens
2. mixed CT disease
3. overlap syndromes

Question 17

def. of vasculitis

Answer 17

inflammation and necrosis of blood vessesl

• dilations and rupture
• ischemia and thrombosis

Question 18

classification of vasculitis

Answer 18

by size

• small
• medium
• large

Question 19

4 constitutional Sx of vasculitis

Answer 19

very unwell

• fever
• weight loss
• anorexia
• fatigue

Question 20

skin manifestations

Answer 20

small 
- pupura
- urticaria
- splinter hemmorage
med
- infacrts
- gangrene

Question 21

other organ involvment

Answer 21

joints - arthritis
kidneys - glomeruloneph and infarcts
brain - strokes and seizures
lungs - hemmorage
heart - MI , myopathy
bowel - infarcts

Question 22

3 tests for vasculitis

Answer 22

1. blood work
   - ESR
   - organ dysfunction
2. tissue biopsy
   - of vessel
3. imaging

Question 23

Sx of giant cell arteritis

Answer 23

• constitutional Sx
• new onset headache
• jaw claudication
• shoulder stiffness
• large and dilated tempral vein

Question 24

what to test for in GCA

Answer 24

1 ESR

2. temporal arteryr biopsy

Question 25

treat of GCA

Answer 25

high does prednisone