Early Diffuse Systemic Scleroderma Causes Worse Outcomes

While there is no cure for patients with systemic scleroderma, there are some therapies to manage symptoms, such as medications, antibiotics, physical therapy, or even surgery. But according to Medical XPress, research from Michigan Medicine shares that patients with early diffuse systemic scleroderma have worse outcomes – including more fatalities and worse organ damage – even with treatment. After evaluating over 300 patients, the researchers found that this particular form of scleroderma led to worse kidney, lung, and heart damage. Interested in learning more? Take a look at the research published in Arthritis Research & Therapy.

The Research

Within the study, researchers sought to understand the clinical characteristics and outcomes associated with early diffuse systemic sclerosis. This rare systemic scleroderma subtype, which occurs most often in those in their 40s and 50s, is characterized by widespread organ problems and skin fibrosis. In their research, the researchers evaluated 301 participants treated over a 9-year period. Of these, many patients received immunosuppressive therapies, considered the standards-of-care. For example, around 66% of patients received Mycophenolate Mofetil, which is often used for scleroderma management.

However, patients with early diffuse systemic scleroderma still saw symptom worsening and disease progression. Even while receiving treatment, 23% of patients lost a significant percentage of forced vital capacity. Patients saw worsening organ fibrosis and skin fibrosis. Additionally, around 6% of patients (approximately 18) passed away during the course of the study.

Ultimately, researchers determined that patients with this scleroderma subtype were more at risk of fatal results and worse disease progression than others, despite available treatment. In the future, researchers believe that therapies such as Nintedanib and tocilizumab could be used as alternative treatments to stop lung fibrosis, maintain lung function, and improve patient outcomes. However, some of these therapies might need to be administered early to prevent complications. As early diffuse systemic scleroderma has one of the highest fatality rates associated with rheumatic diseases, it is necessary to continue research and find ways to better benefit patients.

Systemic Scleroderma

Also known as systemic sclerosis, systemic scleroderma is an autoimmune disorder in which the body’s immune system mistakenly attacks healthy tissue. Most cases are spontaneous, meaning that this is not inherited from family members. This condition results in skin thickening and fibrosis (scar tissue accumulation) on the skin and in various organs. This fibrosis is caused by excess collagen production. While localized scleroderma only affects one area or specific bodily areas, the systemic form can affect the skin and organs. It is 4x more common in females than males. Symptoms include:

  • Skin fibrosis
  • Difficulty swallowing
  • Swelling in the hands and fingers
  • Diarrhea
  • Shortness of breath
  • Heartburn
  • Joint pain
  • Raynaud’s phenomenon
  • High blood pressure
  • Sores on the fingers or painful calcium deposits under the skin
  • Loss of organ function

Learn more about systemic scleroderma.

Jessica Lynn

Jessica Lynn

Jessica Lynn has an educational background in writing and marketing. She firmly believes in the power of writing in amplifying voices, and looks forward to doing so for the rare disease community.

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