Connective Tissue Disease

Question 1

what is Sjogrens syndrome

Answer 1

autoimmune condition characterised by lymphocytic infiltration of exocrine glands

Question 2

what are the symptoms of Sjogrens syndrome

Answer 2

sicca symptoms - dry eyes + mouth arthralgia fatigue vaginal dryness parotid gland swelling

Question 3

what conditions can Sjogrens be secondary too

Answer 3

RA SLE

Question 4

Complications of sjogrens

Answer 4

interstitial lung disease peripheral neuropathy increased lymphoma risk

Question 5

what is diagnosis of Sjogrens based on (3 things)

Answer 5

1. ocular dryness - schrimer’s test 2. positive anti-ro + anti-la 3. typical salivary gland biopsy findings

Question 6

what medication can stimulate saliva production in Sjogrens? what is the main side effect?

Answer 6

pilocarpine - flushing main side effect

Question 7

treatment of arthralgia + fatigue in Sjogrens syndrome

Answer 7

hydroxychlorquine

Question 8

what is systemic sclerosis

Answer 8

multi-system autoimmune disease characterised by microvascular inflammatory damage and fibrosis of skin + internal organs

Question 9

what are the two classifications of systemic sclerosis

Answer 9

limited diffuse

Question 10

differences between limited and diffuse systemic sclerosis

Answer 10

limited - skin fibrosis confined to hands, forearms, face, feet. Late organ involvement diffuse - rapid skin changes, involvement of trunk. Early organ involvement

Question 11

antibody associated with limited systemic sclerosis

Answer 11

anti-centromere

Question 12

antibody associated with diffuse systemic sclerosis

Answer 12

Anti-Scl 70

Question 13

skin features of systemic sclerosis

Answer 13

Calcinosis

Reynauds (often first sign)

Esophageal dysmotility - reflux, dysphagia, strictures

Sclerodactyly

Telangectasia

Question 14

organ involvement in systemic sclerosis

Answer 14

pulmonary hypertension, pulmonary fibrosis

renal AKI

right sided heart failure du to pulmonary HTN

GI malabsorption

Question 15

Treatment of scleroderma in systemic sclerosis

Answer 15

immunosuppression

• cyclophosphamide
• methotrexate
• mycophenolate

Question 16

treatment of reynauds

Answer 16

calcium channel blockers (amlodopine) first line

PDE-5 inhibitors (sildenafil) second line

Question 17

treatment of oesophageal dysmotility in systemic sclerosis

Answer 17

PPI

Prokinetic agent e.g. metoclopramide

Question 18

treatment of renal involvement in systemic sclerosis

Answer 18

ACEi

Question 19

treatment of pulmonary HTN in systemic sclerosis

Answer 19

PDE-5 inhibitor (sildenafil)

endothelin receptor antagonist (bosentan)

Question 20

what antibody is associated with mixed connective tissue disease

Answer 20

Anti-RNP

Question 21

Presentation of anti-pospholipid syndrome

Answer 21

recurrent venous / aterial thrombosis

recurrent fetal loss

Question 22

Anti-phospholipid syndrome increases risk of what?

Answer 22

stroke / MI

Question 23

What type of endocarditis is associated with anti-pospholipid syndrome

Answer 23

Libman- Stacks : endocarditis not caused by bacteria

Question 24

common cutaneous feature of anti-pospholipid syndrome

Answer 24

livedo reticularis

Question 25

antibodies associated with anti-pospholipid syndrome

Answer 25

lupus anti-coagulant anti-cardiolipin

Question 26

treatment of anti-pospholipid syndrome

Answer 26

wafarin low-molecular weight heparin (e.g. dalteparin) in pregnancy

Question 27

what is Ehlers-Danlos syndrome

Answer 27

an inherited connective tissue disease characterised by defective collagen synthesis - autosomal dominant

Question 28

features of ehlers-danlos

Answer 28

joint hypermobility hyperextensible skin tissue fragility - mucosal bleeing/organ rupture

Question 29

what is marfans syndrome

Answer 29

autosomal dominant inhertited connective tissue disease due to defective fibrillin 1

Question 30

features of marfans syndrome

Answer 30

tall, thin stature long fingers hypermonbile joints arthralgia pes planus high arched palate

Question 31

heart murmur in ehlers danlos

Answer 31

aortic regurgitation

Question 32

Cardiac problems in marfans syndrome

Answer 32

dilatation of aortic sinuses - aortic aneursym, aortic regurgitation, aortic dissection mitral valve prolapse