Scleroderma is a rare and chronic condition caused by an abnormal immune response that leads to excessive collagen production. Collagen is a major component of scar tissue.
Too much collagen leads to the characteristic hardening and tightening of the skin seen in scleroderma. In its more severe form, called systemic scleroderma, the internal organs can become damaged and scarred, preventing them from working as they should. This damage can also lead a wide range of related conditions or comorbidities.
There is currently no cure for scleroderma, but there are a number of medications available to manage the disease and its related conditions. These include angiotensin-converting enzyme (ACE) inhibitors, which can be used to treat certain kidney problems and vascular problems, such as high blood pressure or Raynaud’s phenomenon, in scleroderma patients.
What are ACE inhibitors?
ACE inhibitors are a type of medicine that prevents the production of a hormone, called angiotensin 2, by blocking the action of the angiotensin-converting enzyme. This enzyme acts to catalyze the breakdown of the inactive precursor angiotensin 1 into angiotensin 2.
Angiotensin 2 has many roles in the body. It triggers vasoconstriction, or the narrowing of blood vessels, to increase blood pressure. It also causes the release of several other hormones, including norepinephrine, anti-diuretic hormone, and aldosterone. These hormones can further increase blood pressure by altering how the kidneys filter blood. They increase the amount of sodium the kidneys retain, which results in the kidneys reabsorbing more water. More water increases the volume of blood, which in turn again increases blood pressure.
The angiotensin-converting enzyme also breaks down a protein called bradykinin that stimulates vasodilation, or the relaxation and widening of blood vessels. Therefore, ACE inhibitors act in a separate pathway to reduce blood pressure, this time by increasing levels of bradykinin.
In systemic sclerosis, blood levels of angiotensin 2 are usually higher than normal. This can potentially contribute to renal crisis, which is generally preceded by a sudden increase in blood pressure. By reducing levels of angiotensin 2, ACE inhibitors can reduce blood pressure and work to prevent a renal crisis.
ACE inhibitors may be used to treat Raynaud’s phenomenon, as it can cause the blood vessels to dilate and increase blood flow to the hands and other affected areas.
Types of ACE inhibitors
- Lotensin (benazepril)
- Capoten (captopril)
- Vasotec (enalapril)
- Monopril (fosinopril)
- Prinivil or Zestril (lisinopril)
- Univasc (moexipril)
- Aceon (perindopril)
- Accupril (quinapril)
- Altace (ramipril)
- Mavik (trandolapril)
The side effects of ACE inhibitors tend to be mild, and include a dry cough, increased potassium levels in the blood, fatigue, dizziness, headaches, and a loss of taste.
ACE inhibitors should not be used when pregnant.
Scleroderma News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.