Systemic Sclerosis Flashcards
What is the cause of systemic sclerosis?
Unknown
What is systemic sclerosis characterised by?
Hardened, sclerotic skin and other connective tissues
Who is more commonly affected by systemic sclerosis?
Women (4x)
What are the two different patterns of systemic slerosis?
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
What is a subtype of Limited cutaneous systemic sclerosis?
CREST syndrome
What does CREST stand for?
Calcinosis
Raynaud’s phenomenon
oEsophageal dysmotility
Sclerodactyly
Telangiectasia
What is typically the first sign of Limitied Cutaneous systemic sclerosis?
Raynauds syndrome
What is common in people presenting with Limited cutanoeus systmeic sclerosis?
Family history
What antibodies are assoicated with limited cutanoeus systemic sclerosis?
Anti-centromere antibodies
Where on the body does scleroderma affect in someone with limited cutaneous systemic sclerosis?
Face and distal limbs predominately
What is Sclerodactyly?
Bright shiny skin of hands and feet
Fingers can be swollen and painful with reduced mobility and function.
Patients are unable to put palm to palm in prayer sign.
How can diffuse cutaneous systemic sclerosis present?
With the same symptoms of CREST and internal organ dysfunction
Which internal organs tend to be affected in someone presenting with diffuse cutaneous systemic sclerosis?
Kidneys
Lungs
Heart
What cardiovascular problems can diffuse cutaneous systemic scerosis result in?
Hypertension
Coronary artery disease
Pericarditis
What respiratory problems can diffuse cutaneous systemic sclerosis result in?
Pulmonary hypertension
Pulmonary fibrosis.
What renal problems can diffuse cutnaeous systemic sclerosis result in?
Glomerulonephritis
Scleroderma renal crisis.
Which antibodies is diffuse cutaneous systemic sclerosis associated with?
Anti scl-70 antibodies
What is the most common cause of death in someone presenting with diffuse cutanous systemic sclerosis?
Respiratory involvement,
–Interstitial lung disease (ILD)
–Pulmonary arterial hypertension (PAH)
What other antibodies will be present in someone presenting with systemic sclerosis?
ANA positive in 90%
RF positive in 30%
What is the prognosis of diffuse cutaneous systemic sclerosis?
Poor
What medical management can be given for systemic sclerosis?
Nifedipine- Raynauds
ACEi- renal function
Antibiotics- skn infection
What medication should patients with signs of renal disease be started on?
ACEi
