Muscle involvement is one of the most serious symptoms of scleroderma, or systemic sclerosis (SSc), with about one-third of all patients reporting some muscle weakness.
It can have a significant impact on patients quality of life and prognosis, and it can be associated with reduced muscle strength, pain, and difficulty with daily activities.
In some cases, severe muscle involvement can lead to complications like respiratory problems, cardiac involvement, and impaired mobility.
Muscle involvement in scleroderma can be quite varied, with different presentations and degrees of severity making it difficult to not only diagnose properly so that treatments can be provided – but also measuring the activity of muscle involvement is complicated.
Since being diagnosed with Scleroderma back in November 2018 I have constantly had muscle aches and pain which come on in fits and starts 😦
The following article entitled ‘Muscle Involvement in Systemic Sclerosis: A Simple Explanation‘ was written by Professor Susanna Proudman for Scleroderma Australia, and I felt it was quite pertinent, being Scleroderma Awareness Month to highlight this issue here on my blog.
She writes; Systemic sclerosis (SSc, a.k.a. scleroderma), is an autoimmune disease that affects the skin, blood vessels, and various organs including the muscles. Muscle problems in scleroderma can range from mild discomfort to severe weakness that affects daily activities. Understanding how and why muscles are affected can help people manage their symptoms and improve their quality of life.
Muscle problems are more common in people with diffuse systemic sclerosis (dcSSc) than in those with limited systemic sclerosis (lcSSc).
How Does Scleroderma Affect the Muscles?
The overactive immune system in people with scleroderma mistakenly attacks the body’s own tissues. This leads to inflammation and excessive production of collagen, a protein that normally helps keep the skin and organs firm and elastic. In scleroderma, too much collagen builds up, causing thickening and stiffness in various parts of the body, including the muscles.
There are two main ways that scleroderma affects the muscles – Inflammation and Fibrosis.
Inflammation of the Muscles (Myositis): Some people with scleroderma develop a condition called myositis, which means “muscle inflammation.” This can cause pain, tenderness, and weakness, especially in the shoulders, upper arms, hips, and thighs.
Myositis makes it difficult to climb stairs, lift objects, or even stand up from a seated position. Over time, if the inflammation is not controlled, the muscles can become weaker and smaller.
This form improves with immunosuppressive medications.
Fibrosis (Scarring) of the Muscles: In addition to inflammation, scleroderma can cause fibrosis, or scarring, in the muscles. This happens when excess collagen replaces healthy muscle tissue, making the muscles stiff and less flexible.
Fibrosis can make movement difficult and lead to a gradual loss of strength. Unfortunately this form does not respond to immunosuppressive medications.
Symptoms of Muscle Involvement in Scleroderma
People with scleroderma-related muscle problems may experience:
- Muscle weakness, especially in the arms and legs
- Muscle pain or tenderness
- Fatigue, even with mild activity
- Stiffness, particularly in the morning or after rest
- Difficulty with everyday tasks like walking, lifting objects, or getting out of a chair
These symptoms can vary from person to person. Some individuals may have only mild muscle issues, while others may experience severe weakness that affects their independence.
Diagnosing Muscle Involvement in Scleroderma
Doctors use several tests to determine if scleroderma is affecting the muscles. These may include:
- Blood tests to check for muscle damage or inflammation (e.g., creatine kinase levels)
- Electromyography (EMG) to measure the electrical activity of muscles
- MRI scans to detect muscle inflammation or fibrosis
- Muscle biopsy to examine muscle tissue under a microscope which is the most reliable way to confirm the diagnosis.
However, these tests are not perfect. For example, a person can still have muscle problems even if their CK levels are normal. As there is no standard classification system for muscle disease in scleroderma, estimates of how common muscle involvement is in scleroderma vary widely, from 6% to 96% of patients. The proportion of patients enrolled in the Australian Scleroderma Cohort Study with biopsy-proven myositis ranges from 2-10%
Why Is Muscle Involvement Often Missed?
Doctors may not always recognize muscle problems in people with scleroderma. This is because muscle weakness can have other causes, such as
- Lack of physical activity due to pain or stiffness
- Poor nutrition
- Side effects of medications like steroids or cholesterol-lowering drugs
- Skin thickening, joint stiffness, or internal scarring, which can also limit movement
Importance of Early Diagnosis & Screening
It is crucial to identify muscle inflammation, if it is present, early so that treatment with immunosuppressive medications can begin as soon as possible. Without treatment, muscle weakness may worsen over time. Since muscle problems are common in scleroderma and can have a major impact on daily life, some experts believe that all SSc patients should be screened for muscle involvement both at the time of diagnosis and during follow-up visits.
Treatment and Management
There is no cure for scleroderma, but muscle involvement can often be managed with a combination of treatments:
- Medications – Corticosteroids and immunosuppressive drugs can help reduce inflammation and slow muscle damage.
- Physical therapy – Stretching and strengthening exercises can help maintain mobility and prevent stiffness.
- Pain management – Over-the-counter pain relievers, heat therapy, and massage may provide relief.
- Nutrition – A balanced diet with enough protein helps support muscle health.
Muscle involvement in scleroderma can be challenging, but early diagnosis and treatment can help improve symptoms and maintain function. Staying active, following a treatment plan, and working with healthcare professionals can make a big difference in managing this aspect of the disease.
I hope that you’ve found this information helpful.
Blessings ’til next time 🙂
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