Scleroderma: Information on Causes, Diagnosis & Treatments

Autoimmune Connective Tissue Scleroderma is an autoimmune disease involving the skin’s connective tissue where it can thicken and become inflamed. Other characteristics of scleroderma involved spontaneous scarring, blood vessel disease, and an overactive immune system. While scleroderma typically affects just the skin, it can harm other structures in the body including the digestive tract, internal organs, and blood vessels.

Women are more likely to develop scleroderma compared to men, especially between the ages of 30 years and 50 years. No cure has yet been found to treat scleroderma. However, many treatment options can improve the patient’s quality of life and alleviate symptoms. Doctors classify scleroderma as localized, systemic, or widespread when the initial onset of the disease spreads to internal organs.

  • Localized scleroderma has its own two categories as linear (causing streaks or lines of thickened skin on the face, legs or arms) or morphea where oval-shaped hardened patches are detected on the skin.

  • Widespread scleroderma begins in a certain area on the skin and eventually travels to other areas of the body including internal organs.

  • Systemic scleroderma (generalized) usually involves multiple systems or body parts. The systemic kinds of scleroderma are differentiated into two categories including:
    • Limited scleroderma that moves slowly affecting the skin on the feet, hands, and face and causing damage to the esophagus, intestines, or lungs. (CREST syndrome)

    • Diffuse scleroderma that develops quickly by thickening the skin on the feet, hands, upper arms, thighs, and middle part of the body. It can also affect internal organs including the gastrointestinal tract, kidneys, lungs, and the heart.

Who Is at Risk for Scleroderma?

The disease is caused by an overproduction of collagen in body tissue that accumulates over time. Collagen is a fibrous protein that produces connective tissue in the body, including the skin. Scientists have yet to determine why abnormal collagen production occurs. However, it is thought that it is caused by an abnormality in the immune system due to an exposure to solvents, pesticides, and other harmful chemicals, especially in individuals who are genetically predisposed to dangerous chemicals.

While anyone can develop scleroderma, the disease tends to happen more often in females compared to males. Ethnicity is also another factor where some groups of individuals are more likely to develop the condition than others. This includes African-Americans and Choctaw Native Americans.

In addition to the above risk factors, those who acquire the condition can develop mild to severe complications that include:

  • Lungs – Scleroderma can lead to pulmonary fibrosis (scarring of lung tissue) that reduces lung function and the ability to breathe normally. The condition might also lead to pulmonary hypertension where arteries to the lungs can constrict or thicken, causing high blood pressure.

  • Kidneys – Many individuals with scleroderma develop elevated blood pressure when the kidneys are affected because of elevated levels of protein found in the urine. Serious kidney complications associated with the disease could include renal crisis that involves rapid kidney failure and high blood pressure.

  • Fingertips – Scleroderma can cause a form of Raynaud’s disease that in severe cases restricts blood flow and permanently damages the fingertips leading to skin ulcers (sores) and pits. In the most severe cases, amputation may be required due to gangrene.

  • Heart – Scleroderma can cause scarring of heart tissue that leads to arrhythmias (abnormal heartbeats) and congestive heart failure along with pericarditis when the membrane sac around the heart becomes inflamed. Scleroderma on the right side of the heart can increase blood pressure and because the heart muscle to wear out.

  • Digestive Tract– Many individuals with scleroderma have digestive issues which cause difficulty in swallowing and producing acid reflux that leads to bouts of diarrhea followed by constipation.

  • Teeth – If the facial skin becomes severely tightened, it can cause the amount to narrow and reduce in size making it difficult to brush teeth. In addition, scleroderma also restricts saliva causing tooth decay and other problems.

  • Sexual function – Scleroderma affects erectile function in men and sexual function in women by constricting the vaginal opening and decreasing normal sexual lubrication.

Common Symptoms

The common signs and symptoms of scleroderma involve:

  • Skin – Nearly all cases of scleroderma begin with tightening and hardening of the skin in either lines or oval-shaped patches. The doctor will categorize the type of scleroderma by the size of the patch, its location, and numbers. Shiny skin is a symptom of scleroderma where the skin has been restricted in movement and becomes tight.

  • Toes or Fingers – In the initial stages of scleroderma, the body will have an exaggerated response to emotional distress or cold temperatures that lead to color changes, pain, or numbness in the toes or fingers. This condition is referred to as Raynaud’s disease.

  • Digestive Tract – Scleroderma can cause acid reflux where the esophagus becomes damaged. This may lead to problems in absorbing nutrients if the muscles in the intestines cannot properly move food through the digestive tract.

  • Internal Organs – Scleroderma is known to cause significant problems that affect the kidneys, lungs, and heart. Without treatment, these problems can become life-threatening.

Diagnosing Scleroderma

Because scleroderma affects many different parts of the body including the skin and internal organs, it is very challenging to diagnose. However, the doctor will perform a thorough physical examination and recommend blood tests to look for specific antibodies associated with problems in the immune system. A biopsy might also be performed or a small sample of tissue removed from the skin to be examined under laboratory conditions. The pathologist will look for any abnormalities in the skin cells.

Likely, the patient will undergo pulmonary function tests (breathing test) to determine lung capacity and a CT (computerized tomography) scan of the lungs to determine function. The doctor will likely recommend an echocardiogram to better understand the function of the heart and determine if the heart muscle has been affected by scleroderma.

Scleroderma Treatments

Many of the symptoms associated with the disease will disappear without treatment between 3 to 5 years. However, if scleroderma affects the body’s internal organs, the condition can become significantly worse with time. When this occurs, the doctor will likely recommend a variety of treatments that could include:

  • Medications – Medical science has not yet developed a medication that can stop scleroderma when the body over produces collagen. However, numerous drugs can control the symptoms of scleroderma and prevent many of the associated complications. These medications can include:
    • Blood vessel dilating drugs

    • Immune system suppressing drugs

    • Stomach acid reducing drugs

    • Infection preventing drugs including antibiotic appointments and pills

    • Pain relieving medications including prescription drugs and over-the-counter painkillers
  • Therapy – Occupational and physical therapy can help manage pain, maintain the patient’s independence with participating in daily activities and improve their mobility and strength.

  • Surgical Procedures – Surgeries typically the last resort when all other options have failed. This includes amputation or lung transplant when pulmonary hypertension associated with scleroderma has caused extensive damage to the arteries supplying blood to the lungs.

Researchers or finding new treatments all the time to help individuals suffering from scleroderma to live a more active lifestyle and feel better.

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Sources:

https://www.sclero.org/scleroderma/autoimmune/cancer/a-to-z.html

https://my.clevelandclinic.org/health/articles/scleroderma-an-overview

https://www.niams.nih.gov/health-topics/scleroderma

http://www.mayoclinic.org/diseases-conditions/scleroderma/home/ovc-20206014

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