US Pharm. 2022;47(4):44-45.

Scleroderma is an autoimmune connective tissue and rheumatic disease that causes inflammation in the skin and other areas of the body, resulting in hardening and tightening of the skin and connective tissues. Through this process, the body makes too much collagen, causing patches of tight, hard skin.

Scleroderma involves many systems in the body. The following definitions can help better to understand how the scleroderma affects each of those systems: Connective tissue supports, protects, and provides structure to other tissues and organs. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage.1 Autoimmune diseases happen when the immune system, which normally helps protect the body from infection and disease, attacks its own tissues.1 Rheumatic disease refers to a group of conditions characterized by inflammation or pain in the muscles, joints, or fibrous tissue.2

There is no cure for scleroderma. The goal of treatment is to relieve symptoms and stop the progression of the disease. Early diagnosis and ongoing monitoring are very important.1,2

Etiology and Risk Factors

The cause of scleroderma is unknown. However, scientists think that the immune system overreacts and causes inflammation and injury to the cells that line blood vessels. This triggers connective tissue cells, especially a cell type called fibroblasts, to make too much collagen and other proteins. The fibroblasts live longer than normal, causing a buildup of collagen in the skin and other organs, leading to the signs and symptoms of scleroderma.3

Factors that may contribute to the disease are genetic predisposition, environment, immune system, and hormonal differences between women and men.

Anyone can acquire scleroderma, but it is more common in women than in men. The disease usually appears in individuals aged between 30 and 50 years and is more common in adults than children. It can affect people of all races and ethnic groups but is more common in African Americans than European Americans. African Americans with scleroderma develop the disease earlier when compared with other groups.1-3

Scleroderma Types and Symptoms

The symptoms of scleroderma vary from person to person depending on the type of scleroderma.1,4-6

Localized Scleroderma

This type of scleroderma typically causes patches of thick, hard skin in one of two patterns.

Morphea Scleroderma: The more common form of localized scleroderma that most often forms on the abdomen and back. It can also sometimes develop on the face, arms, and legs. Morphea scleroderma can be limited to one to four small patches (plaque morphea), or in some cases, it can spread over large areas of the body (generalized morphea). It most commonly occurs in adults aged between 20 and 50 years but can also occur in children.

Linear Scleroderma: Causes lines of thickened or different-colored skin to run down the arm, leg, and—rarely—on the forehead.

Systemic Scleroderma

Systemic scleroderma, also known as systemic sclerosis, may onset quickly or gradually and may cause problems with internal organs in addition to the skin. Many people with this type of scleroderma have fatigue.

Limited Cutaneous Scleroderma: Gradual onset, usually affects skin on fingers, hands, face, lower arms, and legs below the knees. It can also cause problems with blood vessels and esophagus. The limited form has internal organ involvement but is generally milder than in the diffuse form. People with limited cutaneous scleroderma often have all or some of the following symptoms, sometimes abbreviated as CREST: Calcinosis, Raynaud’s phenomenon (decreased blood flow to the fingers), Esophageal dysfunction, Sclerodactyly, and Telangiectasia.3

Diffuse Cutaneous Scleroderma: Sudden onset, usually with skin thickening on fingers or toes. The skin thickening then spreads to the rest of body above the elbows and/or knees. This type of scleroderma can damage internal organs, such as the digestive system, lungs, kidneys, and heart.

Overlap Syndromes

While patients have clear scleroderma-specific symptoms, they also have symptoms that overlap with other autoimmune diseases including lupus and myositis. An example is mixed connective tissue disorder, which includes symptoms that are common in scleroderma, lupus, and myositis. The specific antibody determines the nature of the overlap syndrome.

Although CREST historically refers to the limited scleroderma, people with the diffuse form of scleroderma can also have CREST features.

Diagnosing scleroderma is easier if some of the primary physical symptoms or signs are present, such as Raynaud’s phenomenon or skin that appears to suddenly become swollen or thick. There is no single test for a diagnosis. It is a clinical diagnosis that requires a thorough examination and history by a rheumatologist.

General Guidelines for Treatment

No treatment is available to date that can stop the overproduction of collagen—a characteristic of scleroderma. However, a variety of treatments can help control symptoms and prevent complications.1-3,7

Since scleroderma affects many different parts of the body, the choice of medication will vary depending on the symptoms.

Suppressing the Immune System: Medications that suppress the immune system, such as those taken after organ transplants (e.g., azathioprine or rituximab), may help reduce progression of some scleroderma symptoms, such as the thickening of the skin or worsening of lung damage.

Dilating Blood Vessels: Blood pressure medications that dilate blood vessels (e.g., hydralazine or minoxidil) may help treat Raynaud’s phenomenon.

Reducing Digestive Symptoms: Medications that reduce stomach acid can help relieve heartburn (e.g., pantoprazole or omeprazole). Antibiotics and medications that help move food through the intestines may help reduce bloating, diarrhea, and constipation.

Preventing Infections: Cleaning and protection against the cold may help prevent the infection of fingertip ulcers caused by Raynaud’s disease. Regular influenza and pneumonia vaccinations can help protect lungs that have been damaged by scleroderma.

Relieving Pain: If OTC pain relievers do not help enough, physicians might suggest stronger prescription medications to control pain (prescription-strength Motrin and Vicodin).

Physical Therapy: Physical or occupational therapists can help patients improve their strength and mobility and maintain independence with daily
tasks. Hand therapy may help prevent hand contractures.

Stem Cell Transplants: These might be an option for people who have severe symptoms that have not responded to more common treatments. If the lungs or kidneys have been severely damaged, organ transplants may be considered.

The National Institute of Arthritis and Musculoskeletal and Skin Diseases is funding and conducting research to find better ways to treat symptoms, prevent organ damage, and improve the quality of life for people with scleroderma. Multidisciplinary research has also provided new clues for understanding the disease, which is an important step toward prevention and treatment.

REFERENCES

1. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Scleroderma. www.niams.nih.gov/health-topics/scleroderma. Accessed January 2022.
2. Mayo Clinic. Scleroderma. www.mayoclinic.org/diseases-conditions/scleroderma/diagnosis-treatment/drc-20351957. Accessed February 2022.
3. Verywell Health. Symptoms of scleroderma. www.verywellhealth.com/scleroderma-signs-symptoms-and-complications-4177037. Accessed February 2022.
4. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc-ILD) Respir Res. 2019;20(1):13.
5. Ferreli C, Gasparini G, Parodi A, et al. Cutaneous manifestations of scleroderma and scleroderma-like disorders: a comprehensive review. Clin Rev Allergy Immunol. 2017;53(3):306-336.
6. The Scleroderma Education Project. Doctor visits. www.sclerodermainfo.org/guide/doctor-visit. Accessed February 2022.
7. Sobolewski P, Maliska M, Wieczorek M, et al. Systemic sclerosis–multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221-233.

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