Systemic sclerosis is a serious and complicated disease.
The name scleroderma is derived from the Greek and means "hard skin" skleros (= hard) derma (= skin). Instead of the term "scleroderma", the term "systemic sclerosis" is increasingly used today. It clarifies that the hardening can not only affect the organ "skin", but all body organs, e.g. also the blood vessels.
With approximately 40 patients per million people, systemic sclerosis is a rare disease. Four times more women are affected than men. The disease often appears in the 3rd to 5th decade of life.
Variants of scleroderma
Systemic sclerosis is characterised by different variants of progression, but they all have one thing in common: There is a hardening of the connective tissue. Sometimes "only" the skin is affected (scleroderma), but the internal organs and blood vessels can also harden and constrict (systemic sclerosis), leading to pulmonary arterial hypertension (PAH), for example. Another consequence of Vascular constriction are very painful ulcers that impair the quality of life, mostly on the fingertips (so-called digital ulcerations). In extreme cases, the tissue can be so severely affected and inflamed that it dies and amputation is necessary.
Scleroderma is not curable, but can be treated in the meantime, so that the course of the disease can be slowed down and sometimes even stopped, i.e. it does not progress. Therefore, the addition "progressive" (= progressing), as it was usual in the past (progressive systemic sclerosis = SSC), is not used today and is simply referred to as systemic sclerosis.
In some sufferers, the changes are only measurable with technical devices and are not even noticed by the sufferer. Other sufferers are clearly affected all over the body and have hardening of the face and fingers, some of which die off. Systemic sclerosis can also be associated with serious symptoms. Respiratory complaintsThe disease can also be associated with serious problems with swallowing and digestion. Because the disease has many variations, it often takes months or years before a patient can be correctly diagnosed.
Causes of scleroderma
The exact causes of scleroderma are still unknown. The trigger of scleroderma is thought to be a Faulty reaction of the immune system (autoimmune disease). The body's own cells (in this case of the connective tissue) are recognised as foreign or defective. The result is an inflammatory reaction in which the connective tissue cells (fibroblasts) multiply excessively, leading to fibrosis (accumulation of connective tissue cells) with a high production of collagen. The collagen accumulation leads to sclerosis (hardening) of the skin and narrowing of blood vessels.
It is still unclear to what extent additional genetic factors or disturbances in the formation of new connective tissue or the regulation of blood vessels are causative. Other factors such as the influence of viral or bacterial antigens, UV light, environmental toxins, sex hormones, drugs and certain tumours could also play a role.
It is certain that the following conditions are important in the development of scleroderma:
- Pathological proliferation of connective tissue (fibrosis)
- Damage to blood vessels (vasculopathy)
- autoimmune disease
- Genetic predisposition
- Increased formation of endothelin
Endothelin is a substance with a strong vasoconstrictive effect that is formed in the inner lining of blood vessels (endothelium). Endothelin plays an important role in the development of vascular damage: it causes vascular constriction and also contributes to the development of fibrosis and inflammation. Various scientific studies have shown that endothelin concentrations in the blood of SSC patients are elevated.
