Connective tissue disease

Question 1

who is lupus more common in?

Answer 1

women of childbearing age

afro-carribean, asian, chinese, hispanic

Question 2

pahogenesis of SLE?

Answer 2

genetic factors cause dysregulated apoptosis and cells are removed less efficiently
APC recognises cell contents as antigen and presents to T/B cells and produce autoantibody
attacks cells- inflamation and forms immune complexes which activate complement and cytokines
depositon in basement membrane and inflammation causes scarring

Question 3

what are the antibodies found in APLS

Answer 3

lupus anticoagulant
anti-cardiolipin
anti-beta2glycoprotein

Question 4

what feature is APLS associated with

Answer 4

venous and arterial thrombosis and recurrent miscarriage

Question 5

antibodies not specific for SLE?

Answer 5

anti-ro - congenital heart block and neonatal lupus in pregnant mothers
anti-ANA

Question 6

antibodies specific for lupus

Answer 6

anti-dsDNA
anti-smith
anti-Sm - most specific but rarely seen

Question 7

cutaneous features of SLE

Answer 7

discoid or subacute photosensitive malar rash
non-scarring alopecia
oral ulceration
acute cutaneous lupus

Question 8

what is jaccouds arthropathy

Answer 8

arthralgia that is reversible with no joint damage

Question 9

whos more likely to get sjogrens

Answer 9

women and middle age

Question 10

sjogrens has an increased risk of what?

Answer 10

lymphoma

Question 11

antibodies in sjogrens?

Answer 11

anti-ro
anti-la
IgG, raises ESR, PV

Question 12

what is diffuse SS and what systemic effects can it typically cause

Answer 12

skin involvement on extremities above and below elbows and knees plus face and trunk
renal crisis and ILD

Question 13

what is limited SS and what systemic effects does it typically cause

Answer 13

skin involvement limited to extremities below elbow and knee and face
pulmonary hypertension

Question 14

pathogeneis of SS

Answer 14

genetic deformity causes vasculopathy leading to dysregulated inflammation and fibrosis . becomes vicious cycle

Question 15

antibodies associated with limited SS

Answer 15

anticentromere

Question 16

antibodies associated with diffuse SS

Answer 16

anti-ScL-70

anti-RNA polymerase

Question 17

facial changes in SS

Answer 17

smaller mouth and puckering
beaked nose
thickened/tighter skin
telangiectasia

Question 18

management of SS induced lung fibrosis

Answer 18

mycophenolate
cyclophosphamide
ritixumab
nintedanib

Question 19

managing pulmonary hypertension in SS?

Answer 19

sildenafil
bosentan
eproprostenol
oxygen

Question 20

triphasic stages of raynaud’s?

Answer 20

blanching
acrocyanosis
reactive hyperaemia

Question 21

management of raynaud’s to prevent digital ulcers?

Answer 21

nifedipine
ARB, nitrates, fluoxetine 
sildenafil 
iloprost 
bosentan if there are ulcers