Scleroderma - A Brief Insight

At the end of my studies, I took on a deep dive into scleroderma.  It's a rare disease, but its complex symptoms make it incredibly fascinating from a physiotherapeutic or osteopathic treatment perspective.

Below, you will find a brief overview of the condition. If you want to dive deeper into the topic or are affected yourself, feel free to read my master's thesis or get in contact with me.

What is Scleroderma?

Scleroderma (from the Greek "skleros" = hard; "derma" = skin) is a connective tissue disorder characterized by the proliferation of collagen fibers, which leads to the hardening of the connective tissue.

Scleroderma Angiokeratoma Mohammad2018, Angiokeratoma meibelli, CC BY-SA 4.0

This autoimmune disease belongs to the collagenosis family, and manifests primarily in two distinct forms:

• The non-life-threatening localized scleroderma, where symptoms are restricted to various areas of the skin and typically resolve spontaneously after a few years

• The far more serious systemic scleroderma, which also attacks internal organs like the heart, lungs, intestines, and kidneys.

Pathophysiology

The source of this vast array of symptoms is microangiopathy—essentially, a disease of the small blood vessels. Think of it as a highly specific type of arteriosclerosis, where the blood vessel walls become rigid and impermeable due to the hyperproliferation of collagen fibers. This leads to vascular diseases (blood vessel disorders) and consequently to organ damage that can ultimately prove fatal, usually due to cardiovascular complications.

In addition to endothelial dysfunction, key drivers of the disease include heightened activity of both the immune system and fibroblasts.

The latter leads to an increased accumulation of collagen fibers in the dermis, resulting in the thickening and hardening of the connective tissue.

Exactly how heavily the immune system is involved in driving the disease—and whether its role is primary or secondary—remains to be fully understood.

However, the latest studies show that three key cell types (endothelial cells, fibroblasts, and T and B lymphocytes) are instrumental in the onset and progression of the disease, stimulating each other through growth factors and cytokines.

Even so, major parts of the pathophysiology remain a mystery and are currently the focus of intensive scientific research.

Physiotherapy and Osteopathy Treatment Options

While these vascular complications are the most serious long-term threats, other symptoms arise that can be highly responsive to physiotherapy or osteopathic care.

Treatments are primarily symptom-focused, aiming to relieve discomfort and, ideally, slow down the progression of the disease.

Here is a snapshot of our therapeutic approaches:

• Reduced lung volume due to decreased chest wall elasticity: Restricted joint mobility can be improved by mobilizing the rib cage and the affected joints 

• Further stretching of calcifying joints (especially in the hands and shoulders): Fascial structures become the foundation of effective treatment

• Reduced skin elasticity: This can also be enhanced through mobilization, stretching, and connective tissue massages

• Gastrointestinal and renal complaints (e.g., acid reflux): Can be alleviated using visceral osteopathic treatments

• Muscular hypertonia and retractions: Can be treated through massage, trigger point therapy, and dry needling

• Patient reconditioning: Achieved through a customized exercise program and targeted training

The scope of further treatment options is vast and easily transferable to many other clinical pictures in rheumatology.

Beyond our therapeutic interventions, the foundation of excellent care relies on comprehensive medical and psychological support. Many patients need time to process the diagnosis and come to terms with the condition.

To get a real-world glimpse into the life of a scleroderma patient and explore this topic in much greater depth, check out my linked final thesis.

Whenever you need us, we are here for you!

Your BodyLab Team, your connective tissue specialists

Osteopathy and Physiotherapy | Rehabilitation and Training

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Cover Image Credits

Leith C Jones at English Wikipedia, MercMorphea, CC BY 3.0, details on Wikimedia Commons