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Scleroderma: What you need to know

What is Scleroderma?

SclerodermaScleroderma (pronounced skler-oh-DUR-muh) is a chronic connective tissue (these are the fibers that provide the framework and support for your body) disease that is generally classified as one of the autoimmune rheumatic diseases. It can cause the tightening and hardening of the skin and the connective tissues.

The word “scleroderma” derives from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Signs and symptoms vary based on the type of scleroderma the patient has, and varies widely from patient-to-patient as with many other autoimmune disorders. Scleroderma is a very common overlap disease for those suffering with lupus. Therefore, we felt it was important to share information on this condition.

There are two main types of this disease, localized and systemic:

Localized often only affects a few places on the skin or muscles, not harming major organs. It can be severe and leave long term skin damage, or it can disappear and get better, this varies between individual patients. Generally, this form of scleroderma is relatively mild with the internal organs not usually affected. Patients with localized scleroderma rarely go on to develop systemic scleroderma. Although most patients can be classified as having diffuse or limited disease, different people may have different symptoms and a different combination of symptoms of the illness.

There are two types of localized scleroderma:

Systemic can affect the skin, the tissue under the skin, as well as the internal structures of the body, such as the esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. The changes that occur in systemic scleroderma may affect the connective tissue in many parts of the body. It can also affect blood vessels, muscles and joints. The tissues of involved organs become hard and fibrous, causing them to function less efficiently. The term systemic sclerosis is also used to describe systemic scleroderma. The word “sclerosis” indicates that hardening may occur in the internal systems of the body.

There are two kinds of systemic (or generalized) scleroderma:

CREST stands for the initial letters of five common features:

  1. Calcinosis- Deposit of calcium in the skin tissues.
  2. Raynaud Phenomenon- This occurs when the fingers lose their blood supply and go numb at the slightest sign of cold.
  3. Esophageal dysfunction- When the valve in the esophagus does not work properly, acids from the stomach can enter the esophagus causing heartburn or acid reflux.
  4. Sclerodactyly- This is a thickening of the skin of the fingers or toes.
  5. Telangiectasia- These are tiny red “spider veins” that typically appear on the hands and face.
3-72dpi-Steps-of-Raynauds

 

*Note- Persons with limited scleroderma, and occasionally those with diffuse scleroderma, can develop pulmonary hypertension, a condition in which the blood vessels in the lungs become narrow. This leads to reduced blood flow through the lungs which results in shortness of breath.

Scleroderma is not contagious, infectious, cancerous or malignant.

Who develops scleroderma?

Here are some facts about who develops scleroderma:

What causes scleroderma?

Although the cause of scleroderma is unknown, doctors do know that collagen is a factor. An overproduction and accumulation of collagen (a fibrous type of protein that makes up the connective tissues of the body, including the skin) can lead to scleroderma. Although doctors aren’t sure what causes this abnormal collagen production, the body’s immune system appears to play a role. Like in other autoimmune diseases like lupus, for unknown reasons, the immune system turns against the body, producing inflammation and in the specific case of scleroderma, the overproduction of collagen.

What are the Signs and Symptoms?

Scleroderma’s signs and symptoms vary from patient to patient, are dependent on which type of scleroderma (see above) has been diagnosed, and what parts of the body are involved. Some of these signs and symptoms are:

How is it diagnosed?

Because scleroderma can take so many forms and affect so many different areas of the body, it can be difficult to diagnose. A rheumatologist (a specialist in arthritis and other diseases that cause swelling in the joints) or a dermatologist (skin doctor) might be the physician that would be recommended by your primary caregiver. This would depend on the symptoms that you are experiencing. Here are some of the things a doctor might use to help diagnose scleroderma:

*Note-You may be referred to other doctors if your symptoms involve the kidneys, heart, digestion, lungs, teeth, speech or movement.

How is it treated?

There are no drugs at this time that stop the underlying process that causes scleroderma, but many medications exist to help control symptoms and prevent further complications. Depending on the presenting symptoms, medications may be prescribed to do the following:

What are some complications of scleroderma?

Medical complications from scleroderma can range from mild to severe depending on the type of scleroderma. Most of the below listed complications arise from systemic scleroderma:

Are there alternative treatments or anything that can prevent/manage it?

There are a number of steps you can take to manage symptoms of scleroderma, these are similar to the guidelines for anyone with chronic autoimmune diseases. Here are a few:

Scleroderma

How do I cope with a diagnosis?

There are many feelings that go along with learning to cope with a chronic illness and they are all normal. You may not know where to turn to for advice, support, or information. Your family, friends, and caregivers may feel the same way.

The first thing to understand is that a person with scleroderma, lupus, fibromyalgia, or any other chronic illness is just that. A person. The disease should not become your identity. There is so much more to each individual than their diagnosis, so maintaining a positive but realistic attitude can really help when learning to live with any chronic disease. Try to lead as full of a life as possible and understand that this is just a part of your life, meaning that the disease is what you have, not who you are. If you are feeling depressed or hopeless, please seek counseling from a professional to help provide the tools to help you cope with these complicated feelings.

Doctor consults a young coupleMaintain good communication with your treating physician(s) and be your own best advocate. Keep organized medical records, bring a support person along to visits when you can to help you digest and remember information and advice, and be open and honest with your doctor, following the recommended protocol.  A network, or team of friends, family, medical providers, support groups, and resources can help you successfully navigate through your disease.

A new diagnosis of scleroderma may feel overwhelming, but it doesn’t have to be. It is a complex disease and the symptoms and severity can vary widely between those diagnosed, depending on which parts of the body involved and how they are affected. If you or someone you care about is showing any of the above signs or symptoms, getting a quick, accurate diagnosis and subsequent treatment, can help to minimize the symptoms of scleroderma, thereby increasing the chances of irreversible damage.

Here are some great resource links from the Scleroderma Foundation: 

Patient ResourcesPatient Brochures

Sources: slceroderma.org, mayoclinic, niams.nih.gov, webmd.com, duanerade.com

*All resources provided by Molly’s Fund are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your medical provider with any specific questions or concerns.

You have just read this blog on scleroderma, you may also be interested in reading these blogs:

Lupus Overlap Diseases

Coping with a Chronic Illness

The Anti-Inflammatory and Paleo Diets

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Article by : Karrie Sundbom

Karrie is the Digital Marketing Manager at Molly's Fund and responsible for innovating content for all of Molly's Funds online communications, creating memes and graphics, writing the MFFL Newsletter and main lupus blogs, as well as developing and managing the content for all of our social media platforms. Connect with Karrie on LinkedIn and Google+ .
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