High blood pressure in the lungs (pulmonary hypertension) is a rare complication of systemic sclerosis, so it is important to have regular tests to check how well your lungs and heart are working and quickly detect any problems. If necessary, pulmonary hypertension can be treated with specific drugs such as Bosentan, Sildenafil or Iloprost, that improve symptoms such as breathlessness.

Two Bosentan tablets are usually taken twice a day (in the morning and evening), although the dosage can sometimes vary. It may be necessary to spend a day in hospital when you start treatment. If your condition has not improved after 8-16 weeks, or if it has worsened, you may be given another treatment, either instead of Bosentan or alongside it.

Bosentan may cause certain side effects, such as increasing the levels of some enzymes in the liver, which can lead to liver damage. It may also cause anaemia in some people. As a result, your liver will need to be monitored once a month for as long as you are taking Bosentan. Fluid retention has also been reported in up to 30% of patients using Bosentan.

Bosentan may not be suitable if:

  • You already have a problem with your liver
  • You are taking Ciclosporin (a medicine that suppresses the immune system)
  • You are pregnant. This type of medication can also interact with hormonal contraception, such as the combined contraceptive pill, so it is better to use an alternative method, such as condoms

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