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Scleroderma - A Brief Insight

August 12, 2018

Scleroderma
Scleroderma
Scleroderma

At the end of my studies, I delved into scleroderma. A disease that is not widespread, but due to its symptoms, is very interesting for physiotherapeutic or osteopathic treatments.

The following provides a small insight into the disease. Anyone who wishes to delve deeper into the subject or is personally affected can feel free to read my master thesis or get in contact with me.

What is Scleroderma

Scleroderma (Greek "skleros" = hard; "derma" = skin) is a connective tissue disease characterized by the proliferation of collagen fibers and the resulting hardening of the connective tissue.

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

The autoimmune disease belongs to the group of collagenoses, with mainly two distinct forms described:

  • The non-life-threatening circumscribed scleroderma with restriction of symptoms to various skin areas, which mostly heal spontaneously after a few years

  • The significantly more dangerous systemic scleroderma with additional involvement of internal organs such as the heart, lungs, intestine, and kidneys.

Pathophysiology

The origin of the vast symptomatic diversity lies in microangiopathies, which are diseases of the small blood vessels. You can imagine it as a special form of arteriosclerosis where the blood vessel walls become rigid and impermeable due to the hyperproliferation of collagen fibers. The consequences are vascular diseases (blood vessel diseases) and thus also organ diseases that can ultimately lead to death, often through cardiovascular complications.

Aside from endothelial dysfunctions, key disease factors include increased immune system activity and the fibroblasts.

The latter lead to increased accumulation of collagen fibers in the dermis area, causing thickening and hardening of the connective tissue.

It is not yet definitively clear how much the immune system contributes to the development of the disease and whether it plays a primary or secondary role.

However, recent studies show that especially three cell types (endothelial cells, fibroblasts, and T and B lymphocytes) are significantly involved in the outbreak and development of the disease, stimulating each other through growth factors and cytokines.

Nevertheless, large parts of the pathophysiology remain unexplained and are currently the subject of scientific studies.

Physiotherapeutic and Osteopathic Therapy Options

In addition to these vascular complications, which in the long run represent the most threatening manifestations, other complaints arise that are usually well treatable and suitable for therapy through physiotherapy or osteopathy.

The treatments are primarily symptom-focused and aim to alleviate complaints and ideally slow down the progression of the disease.

The following is a small insight into our therapy options:

  • Reduced lung volume due to decreased elasticity of the rib cage: impaired joint mobility can be improved through mobilization of the rib cage and the affected joint parts

  • Further stretching of calcifying joint parts (especially in the hands and shoulders area): fascial structures form the basis of effective treatment

  • Reduced skin elasticity: Also improvable through mobilizations, stretches, and connective tissue massages

  • Gastrointestinal and renal complaints (e.g., esophageal reflux): can be alleviated through visceral osteopathic treatment

  • Muscular hypertonus and contractions: Treatable through massage, trigger point therapy, and dry needling

  • Reconditioning the patient: through a tailored exercise program and training

The spectrum of other treatment options is vast and applicable to many other diseases in the field of rheumatology.

In addition to our therapy options, the basis of good treatment is comprehensive medical and psychological support. Many patients need time to process the diagnosis and accept the disease.

An extensive interview to gain a small insight into the life of a scleroderma patient, as well as a more comprehensive engagement with the subject can be found in the linked final thesis.


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Title Image Credits

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


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