Systemic Sclerosis

Question 1

what is systemic sclerosis (SSc)?

Answer 1

• an autoimmune disorder of the connective tissue, characterised by increased fibrosis and vascular changes
• commonly affects the skin (e.g. scleroderma), but can also affect the gastrointestinal, cardiac, respiratory, and renal systems

Question 2

what are the two main types of SSc?

Answer 2

• limited cutaneous SSc (lcSSc)
• diffuse cutaneous SSc (dcSSc)

Question 3

what’s the difference between limited and diffuse cutaneous systemic sclerosis?

Answer 3

lcSSc:
- affects the face + distal limbs
- develops gradually
- often preceded by raynaud’s

dcSSc:
- affects the trunk + proximal limbs
- develops rapidly
- internal organ involvement (e.g. lungs, kindeys, heart)

Question 4

what acronym is used to remember the symptoms of SSc?

Answer 4

CREST

c - calcinosis
r - raynaud’s
e - (o)esophageal dysmotility
s - sclerodactyly
t - telangiectasia

Question 5

what are the symptoms of SSc?

Answer 5

• tight, dry or itchy skin on the hands, feet, face or trunk
• raynaud’s
• puffy fingers
• oesophageal reflux or difficulty swallowing

Question 6

what are the signs on examination of SSc?

Answer 6

• shiny or discoloured skin on the hands, sometimes with no skin folds
• thickened skin
• calcinosis
• ulcers on the fingers
• telangiectasia on the face, hands or mucosal surfaces
• microstomia
• joint contractures
• depigmentation or hyperpigmentation of skin (e.g. salt-and-pepper skin)

Question 7

what are the investigations for SSc?

Answer 7

• BP
• ECG
• urinalysis
• autoantibody (e.g. ANA, anti-centromere, anti-Scl 70, anti-RNA polymerase III)
• FBC
• U&Es
• HRCT
• PFTs

Question 8

what is the non-pharmacological management of SSc?

Answer 8

• education
• smoking cessation
• physiotherapy
• OT
• emollient

Question 9

what is the pharmacological management of SSc?

Answer 9

• raynaud’s (e.g. nifedipine, sildenafil)
• methotrexate
• cyclophosphamide or mycophenolate mofetil
• biologic (e.g. rituximab)
• ? PPI
• ? ACEi
• ? anti-arrhythmic

Question 10

what are the long-term complications of SSc?

Answer 10

• PAH
• pulmonary fibrosis
• oesophageal dysmotility
• oesophageal stricture
• barrett’s oesophagus
• ‘watermelon stomach’
• gastroparesis
• scleroderma renal crisis
• arrhythmia
• pericarditis
• endocarditis