If you’ve been diagnosed as having Scleroderma, some information about its effects on your heart may be helpful for prevention and management.
First, a little background:
The name Scleroderma refers to a group of rare diseases which result from an overproduction and accumulation of collagen.
Collagen is a fibrous type of protein from which the connective tissues, including your skin, are made…and those tissues are what support your body.
- When excess amounts of collagen are produced, the connective tissues harden and tighten.
Although we aren’t sure yet what prompts the abnormal collagen production, the body’s immune system appears to play a role.
In addition, In some people, symptoms may be triggered by exposure to certain types of pesticides, epoxy resins or solvents.
Scleroderma’s signs and symptoms vary, depending on which parts of your body are involved:
- Nearly everyone who has scleroderma experiences a hardening and tightening of patches on their skin in different shapes and sizes.
- These patches may cover wide areas of the trunk and limbs.
- The skin may look shiny because it’s so tight, and movement of the affected area may be restricted.
Fingers or toes: One of the earliest signs of scleroderma is an intolerance for cold temperatures or emotional distress.
- This condition is called Raynaud’s Disease and it may also occur in people without Scleroderma.
Digestive system: In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren’t moving food properly.
- Scleroderma affects more women than men, usually between the ages of 30-50.
While there isn’t a cure yet, there are a number of treatments available to offer symptom relief and improve the quality of life.
Scleroderma And The Damage To Your Heart
The complications of the heart in the patient with scleroderma were first identified in 1926 by a physician/researcher named J. Heine, followed by Dr. S. Weiss, who described 9 cases where it led to congestive heart failure.
- Later on, its role in a progressive fibrosis (the thickening and scarring of the connective tissue of the heart) was identified.
Thanks to them and ongoing research it has now been established that scleroderma can involve the muscle tissues of the heart (the myocardium), the coronary arteries, the pericardium (the membrane that surrounds your heart) and the heart’s conduction system.
Symptoms Of Scleroderma And The Damage To Your Heart
As with any form of heart disease, the symptoms of scleroderma involvement are varied.
It prevents your body from getting enough oxygen-rich blood. The blood backs up into your lungs instead, which causes shortness of breath (even when resting in a horizontal position) and a buildup of fluid.
- If these symptoms become chronic, meaning they are present at all times, the patient may also have ascites, which is the abnormal buildup of fluid in the abdomen, and/or swelling to their leg(s) or feet.
Right Heart Failure affects the ability of the right ventricle to work under the increased load. It is most commonly the result of pulmonary hypertension.
- Pulmonary hypertension (PHT) is high blood pressure in the heart-to-lung system that delivers fresh (oxygenated) blood to the heart while returning used (oxygen-depleted) blood back to the lungs.
This is a common manifestation of scleroderma and a poor sign:
The ability of the right ventricle to function under this increased load determines both the severity of symptoms and survival.
The signs and symptoms of right heart failure by history, echocardiogram, and catheterization are associated with a significantly increased risk of death.
Additionally, a condition called congestive hepatomegaly may be present, which describes a backup of blood in the liver, as a result of heart failure
➡ Sudden cardiac death can occur, more than likely as a result of irregular heart rhythms (arrhythmias) or failure of the right ventricle.
➡ Chronic shortness of breath (dyspnea) and chest pain are usually present as well.
➡ A feeling that the heart is pounding even when the heart is beating in a normal rhythm has also been reported.
The presence of cardiac involvement in patients with scleroderma is often underestimated and a correct diagnosis depends on the methods used.
- Symptoms of cardiac complications are also often attributed to complications of other systems such as the lungs, the musculoskeletal system or conditions related to the esophagus.
Unfortunately, heart involvement from scleroderma has a poor prognosis. Clinical heart disease affected by scleroderma has been associated with a 70% mortality.
Comorbidity, the presence of two chronic diseases or conditions in a patient (such as anxiety and depression in Parkinson’s patients), include:
- High blood pressure (hypertension)
- Sleep disorders
- Breathing problems (sleep apnea)
- Kidney disease and enlargement or fibrosis (thickening and scarring) of the of the left ventricle.
While the influence of scleroderma on the functions of the heart has been known for nearly a century, how prevalent it is and its prognosis in today’s population has only recently been understood.
The good news is that we now have accurate imaging options, better blood tests and procedures to help identify cardiac-related conditions influenced by a diagnosis of scleroderma.
Early detection will allow us to improve quality of life and longevity in patients with cardiac involvement as a result of scleroderma.
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