Systemic Sclerosis

In station 5 you may be asked to assess a patient with a skin problem who is now breathless


History (3 minutes)


  • Take a history about the breathlessness
    • Ask all timing questions: since when, all the time/come and go, getting worse, sudden/gradual onset etc.
    • Ask all respiratory questions g. pleuritic chest pain, wheeze, cough (and sputum colour, amount, consistency), haemoptysis, exercise tolerance
    • Ask all cardiovascular questions e.g. chest pain, orthopnoea, PND, palps, ankle swelling, syncope, presyncope
  • Specific questions for systemic sclerosis:
    • Last lung function test result? Echo result?
    • BP
    • Urine tests- protein in urine? EGFR?
    • Blood in stool, dysphagia, dyspepsia, diarrhoea/constipation/abdo pain/incontinence, ever had endoscopy?
    • Mobility? PE risk factors?
    • Check if patient has raynauds (do your fingers go cold and change colour in cold weather? [white-blue-red] how long before they return to normal? Which fingers? Thumbs too? Toes? Ears? Nose? Does it ever happen in the warm? Any ulcers/infarcts? Since when have you had this?)
    • Ask about joint pains/swelling/stiffness and muscle pains
    • Ask about skin changes: puffy hands, tight thick skin, skin cracking, determine the extent of skin disease (where, above/below elbows/knees, face, trunk), enquire about hand function. How long has the patient had the skin problem?
  • PMH
  • Dx including treatments already tried and currently on
  • Fx
  • Sx


Examine (3 minutes)


  • Examine respiratory system and cardiovascular system
  • 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
    • Feel the temperature, check cap refill and pulses, move skin over hand, press on telangiectasia
    • Check movement and function of hand ?fixed flexion
  • Face – look at mouth, skin, hair, nose
    • Peri-oral furrows, tight skin, microstomia
    • Pinched nose
    • Feels the skin and press on telangiectasia
    • Ask to open mouth and eyes
  • Legs- vasculitis, telangiectasia, ulcers, check toes
  • Extras: feel abdomen, check for proximal myopathy, offer to check BP


ICE + Explanation (2 minutes)


A suggested explanation is as follows:


Your breathlessness could be related or unrelated to your diagnosis of systemic sclerosis. There any many causes of shortness of breath. I need to exclude common things first such as anaemia and infection by blood tests and a CXR.

If it is related to your systemic sclerosis then it could be a lung/heart/kidney or gut problem. It is possible that your systemic sclerosis is now also affecting the lungs. The lungs can become scarred (fibrosis). I’d like to check your BP and oxygen levels today and arrange some lung function tests, a tracing of your heart, an ultrasound of your heart and test your urine. It may be that we need to do a CT scan of your chest.  Depending on these results we will have more of an idea of the cause.  If your systemic sclerosis is now affecting your lungs most patients are stable and treatments are available if it worsens or is severe.


I will let your rheumatologist know.






Systemic sclerosis is a connective tissue disease characterised by autoimmunity, vasculopathy and fibrosis.


Scleroderma = “thick skin”.

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)

Females aged 35-65 are most commonly affected.



  • Onset is gradual
  • It is more common (70%) and milder than DCSS
  • CREST (calcinosis, raynauds, esophageal dysmotility, sclerodactyly, telangiectasia) and arthralgia/arthritis and myopathy
  • Raynauds precedes skin changes by > 1 year
  • Interstitial Lung disease (ILD) can occur after approx. 5-10 years
  • Pulmonary arterial hypertension (PAH) can occur after approx. 10-20 years
  • +ve Anticentromere Ab (high risk PAH)



  • Onset is more acute and can be rapidly progressive
  • It is less common (30%)
  • It is associated with more organ involvement
  • Can get the “CREST” features and arthritis and myopathy
  • Raynauds often occurs simultaneously with skin thickening
  • After 1-5 years can get renal crisis, ILD, PAH, right heart failure
  • AntiRNA-pol III (renal crisis)
  • Anti-Scl70 (ILD)


The ACR/EULAR Classification criteria for Systemic Sclerosis 2013 are not diagnostic criteria
Causes of SOB in SS:


  1. Common causes of SOB in general population- anaemia, infection, heart failure, angina, COPD, asthma, renal dysfunction
  2. Specific to SS
    1. LcSS
      1. Pul HT: reduced DLCO <60% predicted, FVC/DLCO ratio >1.6, raised RV systolic pressure >35-40 mmHg, RV dilatation
      2. PE
    2. DcSS
      1. ILD: reduced lung volumes and DLCO, fibrosis on HRCT
      2. Aspiration pneumonia
  • Pul HT
  1. Don’t forget other causes of SOB in SS
    1. Cardiac- pericardial effusion, myocardial fibrosis causing restrictive cardiomyopathy, arrhythmias, increased coronary artery disease risk, valve disease
    2. Renal crisis- usually HT, grade3 or 4 retinopathy, reduced renal function, urine dip protein and blood, reduced platelets
  • GI: Gastric antral vascular ectasia (GAVE) leading to anaemia, aspiration pneumonia secondary to oesophageal dysmotility, reflux leading to cough
  1. MSK- reduced mobility causing PEs




Bedside- BP, oxygen sats, urine dip

Lung- CXR, pulmonary function tests (restrictive defect), ABG, HRCT

Heart- ECG, echo, right heart catheter, cardiac MRI, BNP

Bloods FBC, U+E, ESR and CRP, clotting, autoantibodies- ANA (90% are ANA positive), anticentromere, antiScl70, antiRNA pol

Renal- U+E, urine dip, urine PCR, BP, USS, biopsy

GI- haematinics, endoscopy (OGD+colon), barium studies, H breath test

MSK- CK, EMG, muscle biopsy

Skin- calculate the modified Rodnan skin score, nailfold capillaroscopy to look for loss of capillary density and abnormal capillaries





Conservative: educate, support, MDT, lifestyle advice

Medical: immunosuppresion e.g. cyclophosphamide, mycophenolate mofetil, azathioprine, methotrexate, ciclosporin, prednisolone, rituximab, anti-TNF. Treat PAH with Ca channel blockers, diuretics, oxygen, iloprost, bosentan, sildenafil



Written by Dr Sarah Kennedy


Resources used include those in the references section of this webpage as well as

Rheumatology Secrets, 3e

by Sterling West