“At first it was difficult for me to accept scleroderma. But then I decided not to see it as an enemy, but as part of me – part of my life."
Person with scleroderma

Monitoring



Keeping scleroderma in check

Your symptoms of scleroderma (also known as systemic sclerosis) may change over time, so regular monitoring with the doctor is important.1 The type of specialist you see, and how often you see them, will depend on your symptoms and how your care is managed.

People recently diagnosed with scleroderma will have check-ups every 3 to 6 months. These may then become less frequent over time (for example, every 6 to 12 months) if your doctor considers your symptoms to be well managed and unlikely to change very much.1

It is important to spot any changes early. Symptoms can help predict the course of scleroderma.2, 3 Serious skin problems, as well as kidney, heart and lung symptoms, may need particular attention.4, 5


The facts

Although some of the information you read can be quite scary, it is important to know that scleroderma only shortens the life span of a small number of people, who have a very severe, complicated form of the disease.6, 7

  • Overall, 70-80% of people survive for more than 10 years from diagnosis. This is increasing every day8
  • Life expectancy is linked to whether or not the internal organs are affected
    • In a study of nearly 400 patients with scleroderma who were followed up for 15 years, 94% of people with the limited cutaneous form of scleroderma, and 86% of those with the diffuse cutaneous form, were alive after 5 years.9 Speak to your doctor if you don’t know what form you have
  • Fibrosis in the lungs, also called interstitial lung disease (or ILD), is very common in people with scleroderma. It can be seen in up to 90% of people with progressive forms of scleroderma when using high resolution medical scans (HRCT); but not everyone gets symptoms 10, 11
    • Severe lung fibrosis affects around 13 to 16% of people. People with severe fibrosis generally find their lungs get gradually worse within the first 2-3 years after their diagnosis2, 7, 9, 12
  • Another lung condition called pulmonary arterial hypertension, or PAH, can also affect around 15% of people. It can affect how long someone with scleroderma lives, but some treatments are available11, 13

Tests

Your doctor will probably ask you to get lung and heart function tests at least once per year.1, 14, 15 Depending on your treatment, they may also perform kidney and liver function tests, along with blood counts and blood pressure.


Get involved in your own monitoring

It’s important to know what’s normal for you, and to let your doctors know when things change. This can help you keep on top of your scleroderma.

You can refer to the symptoms of scleroderma section on this site and write down any new symptoms or any changes that you have noticed. Talk to your doctor about these at your next appointment.

References

  1. BMJ Best Practice. Systemic sclerosis (scleroderma): monitoring. http://bestpractice.bmj.com/topics/en-gb/295/monitoring accessed May 2017.

  2. McNearney T et al. Pulmonary involvement in systemic sclerosis: Associations with genetic, serologic, sociodemographic, and behavioural factors. Arthritis Rheum. 2007;57(2):318-26.

  3. Nihtyanova SI and Denton CP. Autoantibodies as predictive tools in systemic sclerosis. Nat Rev Rheumatol 2010;6:112-116.

  4. Jaeger V et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One 2016;11(10):e0163894.

  5. Lefèvre G et al. Survival and prognostic factors in systemic sclerosis- associated pulmonary hypertension. Arthritis Rheum 2013;65(9):2412-23.

  6. Steen V et al. Changes in causes of death in systemic sclerosis. Ann Rheum Dis 2007;66:940-944.

  7. Steen V et al. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994;66(60):1625-35.

  8. Korn JH. Scleroderma: a treatable disease. Cleveland Journal of Medicine 2003;70(11):954-968.

  9. Nihtyanova SI et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheum 2014;66(6):1625-35.

  10. Schurawitzki H et al. Interstitial lung disease in progressive systemic sclerosis : High-resolution CT vs radiography. Radiography. 1990;176:755-9.

  11. Solomon J et al. Scleroderma lung disease. Eur Respir Rev 2013;22(127):6-19.

  12. Steen V and Medsger T. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000;43(11):2437-2444.

  13. Allanore Y et al. Systemic sclerosis. Nat Rev Dis Primers 2015;1:1-21.

  14. Proudman S et al. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Int Med Journal 2007;37:485-494.

  15. Humbert M et al. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum. 2011;63(11):3522-30.

     

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