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Pain Management: Scleroderma

Scleroderma is a chronic autoimmune disorder that can cause tightening and thickening of the skin, thickening of blood vessels, and damage to internal organs. The disease usually affects adults between the ages of 30 and 50 and affects more women than men.

There are two types of scleroderma: Localized scleroderma and generalized scleroderma. Localized scleroderma affects mainly the skin, while generalized scleroderma is a systemic disease that may affect many parts of the body.

There are two kinds of localized scleroderma:

Morphea: a condition involving hard, oval-shaped patches on the skin. Patches start out red or purple and then turn whitish in the center. Rarely, this type can affect blood vessels or internal organs (generalized morphea).
Linear: a condition involving lines or streaks of thickened skin on the arms, legs or face.

There are two kinds of generalized scleroderma:

Limited scleroderma: Occurs gradually, and affects the skin of the face, hands and feet with possible damage to the esophagus, lungs, or intestines as well.
Diffuse scleroderma: Occurs with a more rapid onset and involves thickening of the skin of the trunk (middle part of body), thighs and upper arms in addition to the hands and feet. Diffuse scleroderma also affects internal organs (such as the heart, lungs or kidneys) more often.

What Causes Scleroderma?

The cause of scleroderma is not known, but it is one of a group of systemic autoimmune diseases in which the body's own immune system appears to contribute to inflammation of skin and other organs. Doctors know that scleroderma causes the body to make too much collagen (a protein) that can result in thickening or tightening of skin and scarring of internal organs. In addition, abnormal spasm and thickening of blood vessels contributes to tissue damage and may cause abnormally elevated pressure in systemic and pulmonary arteries.

What Are the Symptoms of Scleroderma?

Symptoms of scleroderma may affect the skin, joints and other organs and include:

Hardening/thickening of the skin, especially skin of the hands and/or face, causing a shiny and creaseless appearance
Cold sensitivity of fingers with red, white, and blue discoloration (called Raynaud's phenomenon)
Ulcers/sores on finger tips
Small red spots on the face and chest (called telangiectasias)
Puffiness/swelling or pain in fingers and/or toes
Pain or swelling of joints
Muscle weakness
Dryness of eyes/mouth (called SjÃ¶gren's syndrome)
Edema (swelling)
Shortness of breath
Heartburn
Diarrhea
Weight Loss

How Is Scleroderma Diagnosed?

This condition is diagnosed by physical exam, X-ray studies, and blood tests. Evaluation may include tests of the heart, lungs, and esophagus or a possible skin biopsy.

How Is Scleroderma Treated?

Many different treatments are available to help control scleroderma. Medications used to treat the condition include:

NSAIDs (nonsteroidal anti-inflammatory drugs like ibuprofen or aspirin) for inflammation and pain
Steroids and other immune suppressants to decrease inflammation and treat muscle and joint problems or involvement of internal organs
Medications to improve blood flow to the fingers
blood pressure control medication
Medications to treat elevated pressure in the arteries of the lungs
Heartburn medication

Other treatments include:

Exercise to improve overall health
Skin and joint protection
Stress management to help people deal with this chronic disease
If severe organ damage occurs, organ transplantation may be necessary.

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WebMD Medical Reference provided in collaboration with the Cleveland Clinic

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Important Safety Information you should know about HUMIRA® (adalimumab).

Serious infections have happened in patients receiving HUMIRA. These infections include TB (tuberculosis) and infections caused by viruses, fungi, or bacteria that have spread throughout the body. Some patients have died from these infections.

Before starting HUMIRA:

Tell your doctor if you think you have an infection, are being treated for an infection, have signs of an infection (such as a fever, cough, or flu-like symptoms), have any open sores on your body, have warm, red, or painful skin, get a lot of infections or have infections that keep coming back, have or had hepatitis B infection, take the medicine Kineret (anakinra), have TB or have been in close contact with someone who has TB, have lived in an area where TB or histoplasmosis is common, or were born in, lived in or traveled where there is more risk for getting TB. Your doctor should test you for TB before starting HUMIRA. If your doctor prescribes any medicine for the treatment of TB, you should start taking it before starting HUMIRA and take the full course of TB medicine prescribed.

Tell your doctor if you have any numbness or tingling, or have a disease that affects your nervous system such as multiple sclerosis or Guillain-Barré syndrome, have heart failure or other heart conditions, are scheduled for major surgery, are pregnant, become pregnant, plan to become pregnant or are breastfeeding. Tell your doctor if you are allergic to HUMIRA or any of its ingredients or are allergic to rubber or latex. The needle cover of the prefilled syringe and the pen contain dry natural rubber.

Also, tell your doctor if you have recently received or are scheduled for any vaccines. Except for live vaccines, patients may still receive vaccines while on HUMIRA. It is recommended that children with juvenile idiopathic arthritis be brought up to date with all immunizations prior to starting HUMIRA.

After starting HUMIRA:

Call your doctor right away if you get an infection, or any sign of an infection including a fever, feeling very tired, cough, flu-like symptoms, warm, red or painful skin or if you have any open sores on your body. HUMIRA can make you more likely to get infections or make any infection that you may have worse.

Possible side effects of HUMIRA:

Serious side effects, which sometimes lead to death, have happened in patients taking HUMIRA.

- Serious infections. These infections include TB (tuberculosis) and infections caused by viruses, fungi, or bacteria. Your doctor will examine you for TB and perform a test to see if you have TB. If your doctor feels that you are at risk for TB, you may be treated with medicine for TB before you begin treatment with HUMIRA and during treatment with HUMIRA. Even if your TB test is negative your doctor should carefully monitor you for TB infections while you are taking HUMIRA. Patients who had a negative TB skin test before receiving HUMIRA have developed active TB. Tell your doctor if you have any of the following symptoms while taking or after taking HUMIRA: cough, low-grade fever, weight loss, or loss of body fat and muscle.
- Certain types of cancer. There have been cases of certain kinds of cancer in patients taking HUMIRA or other TNF blockers. Patients with RA, especially more serious RA, may have a higher chance for getting a kind of cancer called lymphoma. Some patients receiving HUMIRA have developed types of cancer called non-melanoma skin cancer (basal cell cancer and squamous cell cancer of the skin), which are generally not life threatening if treated. Tell your doctor if you have a bump or open sore that doesn't heal.
- Allergic reactions. Signs of a serious allergic reaction include skin rash, a swollen face, or trouble breathing.
- Hepatitis B virus reactivation in patients that carry the virus in their blood. Tell your doctor if you have any of the following symptoms: feel unwell, poor appetite, fatigue, fever, rash or joint pain.
- Nervous system problems. Signs and symptoms include: numbness or tingling, problems with your vision, weakness in your arms or legs, and dizziness.
- Blood problems. Symptoms include a fever that does not go away, bruising or bleeding very easily, or looking very pale.
- New heart failure or worsening heart failure you already have. Symptoms include shortness of breath or swelling of your ankles or feet, or sudden weight gain.
- Immune reactions including a lupus-like syndrome. Symptoms include chest discomfort or pain that does not go away, shortness of breath, joint pain, or rash on your cheeks or arms that gets worse in the sun.

Call your doctor or get medical care right away if you develop any of the above symptoms. Your treatment with HUMIRA may be stopped.

Common side effects of HUMIRA are: injection site reactions (redness, rash, swelling, itching or bruising), upper respiratory infections (sinus infections), headaches, rash and nausea.

These are not all the side effects with HUMIRA. Ask your doctor or pharmacist for more information.

Information You Should Know About HUMIRA® (adalimumab).

HUMIRA is used to reduce the signs and symptoms of moderate to severe rheumatoidarthritis in adults, may prevent further damage to your bones and joints, and may help your ability to perform daily activities. HUMIRA can be used alone or with methotrexate or with certain other medicines. HUMIRA is used to reduce the signs and symptoms of moderate to severe polyarticular juvenile idiopathic arthritis in children 4 years of age and older. HUMIRA can be used alone or with methotrexate or with certain other medicines. HUMIRA is used to reduce the signs and symptoms of psoriatic arthritis in adults, may prevent further damage to your bones and joints, and may help your ability to perform daily activities. HUMIRA can be used alone or with certain other medicines. HUMIRA is used to reduce the signs and symptoms of ankylosing spondylitis in adults. HUMIRA is used to reduce the signs and symptoms of moderate to severe Crohn's disease in adults who have not responded well to conventional treatments. HUMIRA is also for these adults with moderate to severe Crohn's disease who have lost response or are unable to tolerate infliximab. HUMIRA is used to treat moderate to severe chronic (lasting a long time) plaque psoriasis in adults who are under the ongoing care of a physician, have the condition in many areas of their body and who may benefit from taking injections or pills (systemic therapy) or phototherapy (treatment using ultraviolet light alone or with pills).

HUMIRA is taken by injection.

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