Connective Tissue Disease

Question 1

how does sjogren’s syndrome present? (4)

Answer 1

dry eyes, mouth + throat
parotid gland enlargement
joint pains
fatigue

Question 2

who is a typical sjogren’s syndrome patient?

Answer 2

middle aged female

Question 3

what antibodies are seen in sjogren’s syndrome?

Answer 3

anti Ro

anti La

Question 4

besides antibodies, what else can be seen in sjogren’s syndrome?

Answer 4

raised IgG

raised inflammatory markers

Question 5

what are patients at increased risk of in sjogren’s syndrome?

Answer 5

lymphoma

Question 6

what is another name for systemic sclerosis?

Answer 6

scleroderma

Question 7

what characterises systemic sclerosis?

Answer 7

vasculopathy
autoimmunity
fibrosis

Question 8

how does systemic sclerosis present? (5)

Answer 8

raynaud's
GORD
telangiectasia 
SOB 
difficulty swallowing

Question 9

what are four red flags for systemic sclerosis?

Answer 9

raynaud’s coming on in mid adulthood
digital ulcers or ischaemia
loss of dexterity in the hands
raynaud’s + SOB

Question 10

what are the two types of systemic sclerosis?

Answer 10

diffuse

limited

Question 11

what is diffuse systemic sclerosis?

Answer 11

when the skin involvement is on extremities above + below the elbows and knees, and the face and trunk

Question 12

what is limited systemic sclerosis?

Answer 12

when the skin involvement is only below the elbows and knees and on the face

Question 13

what five antibodies can be seen in systemic sclerosis?

Answer 13

anti centromere 
anti SCL 70 
anti pm scl 
anti to
anti RNA polymerase

Question 14

what is another name for anti SCL 70?

Answer 14

anti topoisomerase

Question 15

what facial changes can be seen in systemic sclerosis?

Answer 15

small mouth with puckering
beaked nose
thickened and tight skin
telangiectasia

Question 16

what are three possible GI complications of systemic sclerosis?

Answer 16

GORD
malabsorption
small intestinal bacterial overgrowth

Question 17

what are two possible respiratory complications of systemic sclerosis?

Answer 17

interstitial lung disease

pulmonary arterial hypertension

Question 18

what renal complication can occur in systemic sclerosis?

Answer 18

scleroderma renal crisis

Question 19

how does scleroderma renal crisis present?

Answer 19

worsening renal function

sustained high BP

Question 20

what antibody is associated with scleroderma renal crisis?

Answer 20

anti RNA polymerase

Question 21

what puts patients at risk of scleroderma renal crisis?

Answer 21

high dose steroids

Question 22

how is scleroderma renal crisis managed?

Answer 22

ACE inhibitors

Question 23

what are the three phases of raynaud’s phenomenon?

Answer 23

blanching - white
acrocyanosis - blue
reactive hyperaemia - red

Question 24

what is the first line treatment for raynaud’s?

Answer 24

calcium channel blockers - nifedipine

Question 25

how is skin fibrosis in scleroderma managed?

Answer 25

methotrexate

mycophenolate

Question 26

what is the pathophysiology behind SLE?

Answer 26

immune system attacks cells causing inflammation

immune complexes form and precipitate in areas, resulting in further immune response

Question 27

who is most commonly affected by SLE and when does it usually present?

Answer 27

more common in females

commonly presents in childbearing years

Question 28

what diagnostic criteria is used to diagnose SLE?

Answer 28

EULAR/ACR 2019 SLE classification citeria

Question 29

what criteria must be met to diagnose SLE?

Answer 29

ANA positive and score >10 in classification criteria

Question 30

name two markers of SLE

Answer 30

low complement levels

anti phospholipid antibodies

Question 31

what score can be used to monitor SLE activity?

Answer 31

SLEDAI score