Connective tissue disorders

Question 1

major patho of SLE

Answer 1

• autoantibody production
• deposition of immune complexes
• complement activation
• tissue destruciton/vaculitis

Question 2

clinical features of SLE

Answer 2

fatugue most common
4 or more of
Malar rash
Discoid rash
Serositis (pleuritis, pericarditis)
Oral ulcers
Arthirits
Photosensitivity
Blood disoders (cytopenias)
Renal (proteinuria or cell casts)
ANA - high
Immunologic - dsDNA, anti-smith
Neuro- seizure or psychosis without good reason

Question 3

SLE diagnositic tests

Answer 3

1. ANA - sens but not specific
2. anti DSDNA
3. Anti-smith
4. anti-histone
5. pos. LE

Question 4

7 steps to Tx

Answer 4

1. avoid sun
2. NSAIDS
3. local or systemic steroids
4. systemic steroids for severe manifestations
5. anti-malarials - hydroxychloroquine
6. cytotoxic agents - cyclophosphamide

Question 5

main patho in scleroderma

Answer 5

cytokine stim fibroblast which lay down large amounts of collagen

Question 6

2 main types of scleroderma

Answer 6

diffuse and limited

- see P 240 for differnce

Question 7

6 main clincial features by system

Answer 7

1. raynauds
   - initial presentation
2. cutaneous fibrosis
   - sclerodactyl and calcinosis of digits
3. GI
   - dysphaia/reflux (strictures), delayed emptying, constipation/dishhrea
4. pulmonary
   - fibrosis - most common cause of death
5. cardiac involvement
   - effusions, myocardial involvment
6. renal

Question 8

CREST syndrome

Answer 8

type of local scleroderma
Calcinosis of digits
Raynauds
Esoph strictures
Sclerodactyl
Telantectasias

Question 9

Dx tests for scleroderma

Answer 9

1. almost all have high ANA
2. anticentromere very specific for limited form
3. barium swallow for strictures

Question 10

Tx of scleroderma

Answer 10

no good one

1. NSAIDs for pain
2. H2 for GERD
3. treat pulm and renal as they come

Question 11

underlying patho of sjorgens

Answer 11

lymphocytes attack lacrimal and salivary glands

- most women

Question 12

2 types of sjorgens

Answer 12

1. primary
   - dry eyes and mouth and no other disease
2. secondary
   - dry eyes and mouth allong with another CT disease

Question 13

4 main features of sjorgens

Answer 13

1. dry eyes
2. dry outh
3. arthralgias, artrirtis, fatigue
4. other extraglandular manifestations

Question 14

Dx tests

Answer 14

1. ANA in 95, RF in 50-75%

2. Ro SSa in50% and RoSSB in 40%

Question 15

Tx for sjorgens

Answer 15

1. pilocarpine
2. artificial teaers
3. oral hygene
4. NSAIDS, steroids for athritis
5. for secondary, threat other CT disease