Definition of scleroderma
Scleroderma is a chronic disease, discovered in the 1930s, which still continues to generate confusion and doubts.
Scleroderma is also called progressive systemic sclerosis; its name derives from the ancient Greek, whose literal translation is “hard skin”: in fact, this pathology affects the skin causing hardening and thickening of the skin. The areas most affected by scleroderma are the arms, legs and skin around the mouth; however, it can also extend to capillaries, arterioles and internal organs (heart, kidneys, intestines and lungs). In the latter case, the disease could have very serious consequences, up to the death of the subject.
For further information: Scleroderma Symptoms
In Italy, scleroderma affects about 70,000 people, 90% of whom are women: it generally occurs between 40 and 50 years of age, but the most severe form occurs between 20 and 25 years.
Causes
It seems strange that scleroderma affects such a large number of people and, at the same time, the triggering causes have not yet been found: modern research has, however, come to the conclusion that the genetic predisposition of each individual subject, combined with its heritage genic, represent two fundamental factors for the manifestation of the pathology. It should be emphasized that systemic sclerosis is not considered a hereditary disease: in fact, affected individuals have no family members who suffer from this disorder, although it is likely that one or more relatives manifest other autoimmune diseases.
For a long time it was considered an infectious disease: with current research, it is considered "unlikely" that an "infection could trigger a pathology of this type. Despite what has been stated, it has been hypothesized that a virus (cytomegalovirus or CMV, which enters cells reproducing in a parasitic way and sometimes causing death) could be implicated in the origin of scleroderma, being one of the presumed responsible for the activation of the immune response against organs or tissues of the subject.
It has been found that some synthetic substances, such as vinyl chloride, aromatic hydrocarbons and epoxy resins, can cause fibrosis similar to that of a person with scleroderma.
Classification
Scleroderma is classified into two different types; for each of them, there are sub-categories that are distinguished on the basis of the manifestations.
- Systemic scleroderimia - or systemic sclerosis - which can be: limited, diffuse, overlap or CREST. It is “limited” if the thickening and hardening of the skin occurs at the extremities; it is “widespread” if it involves the extremities, face, trunk and internal organs. Sclerodemia is "overlap" in the event that the disorders are also different and typical of the pathologies affecting the connective tissue. By "CREST" we mean a scleroderma pathology characterized by calcinosis, Raynaud's syndrome, disorders of the esophagus, sclerodactyly and telangiectasias.
Being a systemic disease, other organs and systems are also affected: cardiac, respiratory, musculoskeletal, urinary and gastrointestinal systems.
- Localized scleroderma of the linear type (manifested by linear scars on the face, arms, or legs) or morphea (characterized by a sort of thick and hard skin plates).
Generality
Among the cells involved in scleroderma pathogenesis are fibroblasts, endothelial cells of vessels and small arteries, and defense cells (T lymphocytes and B lymphocytes). Collagen fibers are hyperactive: they produce an enormous amount of collagen, which creates clusters; consequently the skin becomes thicker. The skin appendages disappear, as does the hydrolipidic film.
In scleroderma alterations occur in the vessels of the microcirculation, with sclerotic manifestations on the heart, lungs, intestines and skin. An excessive amount of antibodies is produced.
Possible treatments
For further information: Drugs for the treatment of scleroderma
It is not correct to speak of a "cure" for scleroderma: rather, the term "treatment" should be used, which does not completely solve the problem, but can alleviate the damage caused to the patient, improving, in some way, his living conditions .
Among drugs, those capable of delaying fibrosis of tissue, skin or internal organs should be preferred: for example, interferon gamma, penicillin isomer D, kinase inhibitor imatinib. Also calcitriol, a derivative of vitamin D, it could alleviate ailments.
Recently, thalidomide and some prostaglandin agonists have also been considered.
Some associations in the national territory (AILS, the Italian Association for the Fight for Scleroderma, and GILS, the Italian Group for the Fight against Scleroderma) are encouraging fundraising and strengthening scientific research to find a solution to the scleroderma problem as soon as possible.
