connective tissue disorders Flashcards
what are the different types of CTD?
SLE
Sjogren’s syndrome
systemic sclerosis
anti-phospholipid syndrome
what type of hypersensitivity reaction is SLE?
type III
who does SLE typically affect?
women more than men
non-whites
how does SLE usually present?
malar rash
raynaud’s
oral ulcers
systemic inflammation
pleural effusion
what is raynaud’s?
vasospasm of blood vessels in fingers and toes
painful on exposure to the cold in these areas
go white or blue
what investigations are used in SLE?
autoantibodies
FBC - anaemia, leukopenia, thrombocytopenia
urinalysis - glomerulonephritis
what antibodies are seen in SLE?
anti-dsDNA
antiphospholipid
what is the management of SLE?
monitor
mild = hydroxychloroquine
moderate = hydroxychloroquine + oral steroids
severe (organ involvement) = cyclophosphamide + IV steroids
what type of hypersensitivity reaction is Sjogren’s?
type IV
who does Sjogren’s usually affect?
women in 40s
can be secondary to RA or SLE
what is Sjogren’s?
lymphocytic infiltrates in exocrine glands
dryness everywhere
how does Sjogren’s typically present?
dry eyes/mouth/throat
bilateral parotid gland enlargement
joint pain
bad teeth
what investigation is used in Sjogren’s?
Schirmer’s test (filter paper at lower eyelid)
what antibodies are seen in Sjogren’s?
anti-ro
anti-la
what is the management of Sjogren’s?
lubricate eyes
pilocarpine (stimulates saliva)
hydroxychloroquine (arthralgia/fatigue)
what is systemic sclerosis?
autoimmune disorder or stiff, hardening tissue (systemic fibrosis)
why does systemic fibrosis occur?
fibroblast activation + excess collagen deposition
who does systemic sclerosis usually affect?
women 30s-50s
what are the 2 types of systemic sclerosis?
diffuse - visceral organ involvement
limited - CREST
how does diffuse systemic sclerosis present?
renal, GI, heart, joints, lungs affected
pulmonary hypertension + ILD
scleroderma renal crisis
what medication reverses a scleroderml renal crisis?
ACE inhibitors
how does limited systemic sclerosis usually present?
CREST
Calcinosis - calcium deposits in subcutaneous tissue
Raynaud’s
Oesophageal dysmotility - difficulty swallowing, reflux
Sclerodactyly - fibrosis of skin, hands (shiny, puffy hands)
Telangiectasia - skin lesions from dilated capillaries
what investigations are used in systemic sclerosis?
organ screening (renal and lung changes)
what antibodies are seen in systemic sclerosis?
anti-centroemere
anti-Scl-70
what is the management of symptomatic systemic sclerosis?
raynaud’s = CCB
renal = ACEi
reflux
PPI
ILD = immunosuppression
what is anti-phospholipid syndrome?
autoimmune disorder where immune system produces antibodies that attack normal proteins in blood - leads to increased risk of blood clots
who is most at risk for anti-phospholipid syndrome?
people with APL in plamsa
women > men
how does anti-phospholipid syndrome present?
venous and arterial thrombosis (strokes, DVTs and recurrent miscarriages, livedo reticularis)
what is seen on investigation of anti-phospholipid syndrome?
thrombocytopenia
APTT prolongation
immunology
what is the management of anti-phospholipid syndrome?
anti-coagulation
what anti-coag is given to pregnant patients or patients who want to get pregnant?
LMWH
warfarin is teratogenic
