Connective Tissue Disease Flashcards
how does sjogren’s syndrome present? (4)
dry eyes, mouth + throat
parotid gland enlargement
joint pains
fatigue
who is a typical sjogren’s syndrome patient?
middle aged female
what antibodies are seen in sjogren’s syndrome?
anti Ro
anti La
besides antibodies, what else can be seen in sjogren’s syndrome?
raised IgG
raised inflammatory markers
what are patients at increased risk of in sjogren’s syndrome?
lymphoma
what is another name for systemic sclerosis?
scleroderma
what characterises systemic sclerosis?
vasculopathy
autoimmunity
fibrosis
how does systemic sclerosis present? (5)
raynaud's GORD telangiectasia SOB difficulty swallowing
what are four red flags for systemic sclerosis?
raynaud’s coming on in mid adulthood
digital ulcers or ischaemia
loss of dexterity in the hands
raynaud’s + SOB
what are the two types of systemic sclerosis?
diffuse
limited
what is diffuse systemic sclerosis?
when the skin involvement is on extremities above + below the elbows and knees, and the face and trunk
what is limited systemic sclerosis?
when the skin involvement is only below the elbows and knees and on the face
what five antibodies can be seen in systemic sclerosis?
anti centromere anti SCL 70 anti pm scl anti to anti RNA polymerase
what is another name for anti SCL 70?
anti topoisomerase
what facial changes can be seen in systemic sclerosis?
small mouth with puckering
beaked nose
thickened and tight skin
telangiectasia
what are three possible GI complications of systemic sclerosis?
GORD
malabsorption
small intestinal bacterial overgrowth
what are two possible respiratory complications of systemic sclerosis?
interstitial lung disease
pulmonary arterial hypertension
what renal complication can occur in systemic sclerosis?
scleroderma renal crisis
how does scleroderma renal crisis present?
worsening renal function
sustained high BP
what antibody is associated with scleroderma renal crisis?
anti RNA polymerase
what puts patients at risk of scleroderma renal crisis?
high dose steroids
how is scleroderma renal crisis managed?
ACE inhibitors
what are the three phases of raynaud’s phenomenon?
blanching - white
acrocyanosis - blue
reactive hyperaemia - red
what is the first line treatment for raynaud’s?
calcium channel blockers - nifedipine
how is skin fibrosis in scleroderma managed?
methotrexate
mycophenolate
what is the pathophysiology behind SLE?
immune system attacks cells causing inflammation
immune complexes form and precipitate in areas, resulting in further immune response
who is most commonly affected by SLE and when does it usually present?
more common in females
commonly presents in childbearing years
what diagnostic criteria is used to diagnose SLE?
EULAR/ACR 2019 SLE classification citeria
what criteria must be met to diagnose SLE?
ANA positive and score >10 in classification criteria
name two markers of SLE
low complement levels
anti phospholipid antibodies
what score can be used to monitor SLE activity?
SLEDAI score
