Systemic scleroderma is a rare complex multisystem autoimmune disease that causes your body to make excess collagen, which causes scarring of internal organs and skin as well as blood vessel narrowing. There is no cure for this connective tissue disease.
The experts at Rheumatology and Allergy Institute of Connecticut are on the cutting edge of research and treatment for scleroderma. Our team can work with you to create a personalized treatment plan that aims to prevent complications and provide symptom relief.
How scleroderma affects your body
Scleroderma occurs on a spectrum that varies from person to person. Some cases are severe, while other cases are mild. Skin and internal organ scarring and blood vessel narrowing are two major characteristics of the disease. We do everything possible to prevent complications, as scarring can lead to irreversible organ damage.
Scleroderma often starts with episodes of rapid decrease of blood flow to the hands, known as Raynaud phenomenon. In some cases, this is the only symptom for an extended period of time.
Scarring of the lungs and esophagus can make swallowing and breathing difficult, while kidney involvement can result in sudden renal failure. Because no two scleroderma cases are alike, recognizing your disease subtype, stage, and implicated organs is critical in establishing the appropriate course of treatment.
Treatment options for scleroderma
Current treatments target the four major aspects of the disease: inflammation, autoimmunity, vascular disease, and tissue fibrosis. Your provider works with you to choose the best treatments for you. Here are some common treatments for managing scleroderma.
There are two basic forms of inflammation associated with scleroderma. The first is a more common form that can cause arthritis (joint inflammation), myositis (muscle inflammation), or serositis (inflammation of the lining of the heart or lung).
This form of inflammation responds to nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids. The duration of therapy and the dosage of medication are determined by your circumstances. Some patients require ongoing medication, while others recover after a brief course of therapy.
The other type of inflammation involves skin and other tissue damage. This stage of the disease does not respond to NSAIDs or corticosteroids.
This is the most widely used method of managing the inflammatory phase of scleroderma. The idea is that the inflammation is caused by an autoimmune mechanism, with tissue damage and fibrosis as a result. In this approach, fibrosis is an "innocent bystander" triggered by the immune system's cytokines (chemical messengers).
Examples of immunosuppressive medications for managing scleroderma include:
- Antithymocyte globulin
- Mycophenolate mofetil
Your provider discusses the benefits and drawbacks of immunosuppressive drugs with you so you understand how they may affect you.
Vascular drug therapy
Scleroderma can cause widespread issues with medium and small arteries. Vascular drug therapy is critical to controlling vascular disease and preventing specific organ damage. There are three primary aspects of vascular illness that may require treatment: blood vessel spasms, blood vessel thickening, and blood clots.
Vasodilator therapy with medications such as calcium channel blockers is the most effective treatment for blood vessel spasms. Calcium channel blockers improve blood flow to the skin and heart; angiotensin converting enzyme (ACE) inhibitors help reverse the vasospasm of the scleroderma renal crisis; and endothelin-1 receptor inhibitors improve blood flow in the lungs.
A dedicated scleroderma program is crucial for controlling the disease and keeping you as healthy as possible.
If you or a loved one is diagnosed with scleroderma, give us a call to schedule a visit with a Rheumatology and Allergy Institute of Connecticut, LLC, provider. Our office is located in Manchester, Connecticut. Call or book online today.