Scenario - SS
skin changes and breathlessness
Hx - SS
o Skin changes - puffy hands, tight thick skin, skin cracking, determine the extent (where, above/below elbows/knees, face, trunk),
o Hand function, joint pains/swelling/stiffness and muscle pains
o raynauds (do your fingers go cold and change colour in cold weather? [white-blue-red] how long? Which fingers? Thumbs too? Toes? Ears? Nose? Does it happen in the warm? Any ulcers/infarcts?
o Resp – SOB, cough, haemoptysis
o Cardio – CP, palpitations, blackouts
o GI - Blood in stool, dysphagia, dyspepsia, diarrhoea/constipation/ abdo pain/incontinence
PMH/DHx/Allergies/Smoking/alcohol; social/FHx/travel; concerns
Exam - SS
- hands
- face
- eyes
- mouth
- feet
- CVS
- Resp
Exam findings - SS
o Hands- skin, nails, pulps, fingers, elbows, arms; Tight shiny thick skin (sclerodactyly), telangiectasia, bruising, calcinosis, pulp atrophy and pits, digital ulcers/infarcts, atrophic nails, swollen extremities; temperature, cap refill and pulses, move skin over hand, press on telangiectasia
o Check movement and function of hand ?fixed flexion (prayer/reverse; fist, straight out)
o Face – look at mouth, skin, hair, nose; Peri-oral furrows, tight skin, microstomia; beaked nose; Feel the skin and press on telangiectasia; Ask to open mouth and eyes
o Legs- vasculitis, telangiectasia, ulcers, check toes
o CVS + Resp
o Extras: feel abdomen, check for proximal myopathy, offer to check BP
Ix - SS
o Bedside- BP, oxygen sats, urine dip
o Bloods FBC, U+E, ESR and CRP, clotting,
o Autoantibodies - ANA positive (in 90%); Anti‐centromere antibody = limited (in 80%); Scl‐70 antibody = diffuse (in 70%)
o Hand radiographs: calcinosis
Ix - complications - SS
o Lung- CXR, pulmonary function tests (restrictive defect – LL fibrosis), ABG, HRCT
o Heart (fibrosis and arrhyth.)- ECG, echo, right heart catheter, cardiac MRI, BNP
o Renal- U+E, urine dip, urine PCR, BP, USS, biopsy
o GI (dysmotility and malabsorption)- haematinics, endoscopy (OGD+colon), barium studies, H breath test
o MSK- CK, EMG, muscle biopsy
o Skin- calculate the modified Rodnan skin score, nailfold capillaroscopy to look for loss of capillary density and abnormal capillaries
Tx - SS
o Slow progression, manage sx
o Immunosupression
o Raynaud’s therapy - Gloves, hand‐warmers, etc., CCB, prostacyclin infusions
o Resp – Nintedanib for IPF
o Renal - ACE‐Is: prevent hypertensive crisis, reduce mortality from renal failure
o gastrointestinal - Proton‐pump inhibitor for oesophageal reflux
SS - types
o Systemic sclerosis can be localised cutaneous (affecting face, neck, skin below elbows and knees) or diffuse cutaneous (affecting skin proximal to elbows and knees and trunk)
o Females aged 35-65 are most commonly affected
LCSS
o Onset is gradual
o It is more common (70%) and milder than DCSS
o CREST (calcinosis, raynauds, esophageal dysmotility, sclerodactyly, telangiectasia) and arthralgia/arthritis and myopathy
o Raynauds precedes skin changes by > 1 year
o Interstitial Lung disease (ILD) can occur after approx. 5-10 years
o Pulmonary arterial hypertension (PAH) can occur after approx. 10-20 years
o +ve Anticentromere Ab (high risk PAH)
DCSS
o Onset is more acute and can be rapidly progressive
o It is less common (30%)
o It is associated with more organ involvement
o Can get the “CREST” features and arthritis and myopathy
o Raynauds often occurs simultaneously with skin thickening
o After 1-5 years can get renal crisis, ILD, PAH, right heart failure
o AntiRNA-pol III (renal crisis)
o Anti-Scl70 (ILD)
