Different forms of systemic scleroderma differ significantly in the mechanisms of development of pathological reactions, clinical manifestations and prognosis. There is no generally accepted classification of systemic scleroderma, at the same time, the classifications of this disease in different countries have much in common. There are such main clinical forms of systemic scleroderma: diffuse and limited. Read more about the forms of systemic scleroderma further on estet-portal.com.
Features of the diffuse form of systemic scleroderma
The most common are diffuse and limited forms of systemic scleroderma. Signs of a limited form, read further on estet-portal.com. Already in the debut, there are clear differences between these forms of the disease. Thus, the diffuse form of systemic scleroderma has an acute onset and a rapidly progressive course. Often, skin involvement begins at about the same time (during the first year) or earlier. An extensive skin lesion begins with the fingers and spreads along the limbs above the elbow and knee joints, also capturing the skin of the neck, face, décolleté, chest, abdomen and other parts of the body.
In the debut of diffuse form of systemic scleroderma, arthritis often occurs, myositis,periarticular contractures quickly form.
The active formation of the symptom complex, including visceritis, occurs quickly, and the diagnosis can be established already in the first year of the disease. The prognosis for the diffuse form is worse than for the limited one, because with this form of the disease, vital organs are quickly affected - usually in the first 2–4 years: the heart, lungs, kidneys and gastrointestinal tract. The five-year survival rate is 50-60%.
Feature of limited form of systemic scleroderma
In the limited form of systemic scleroderma, the onset of the disease is monosymptomatic, the first symptoms of skin lesions of the hands, feet and face appear on average 5 years after the onset of the systemic reaction. The clinic is dominated by vascular disorders. Sclerotic lesion of internal organs is expressed moderately. Ischemic and trophic changes on the fingers (scars and ulcerations, a decrease in the volume of soft tissues of the distal phalanges, osteolysis) gradually join vasospastic disorders.
Characteristics of the limited form of systemic scleroderma:
- A detailed picture of the disease is formed on average 5 years after the onset of the first symptom.
- At this stage, soft tissue calcification, osteolysis of the nail phalanges appear, the number of telangiectasias increases, in about 6–10% of cases pulmonary arterial hypertension.
- In capillaroscopy, the characteristic feature is dilatation of the capillaries.
- The limited form occurs in 2/3 of patients with SJS, has a chronic, slowly progressive and relatively benign course.
- The five-year survival rate for patients with this form of systemic scleroderma is 85–90%.
What are the features of the cross form of systemic scleroderma?
The Russian school of rheumatology regards the cross form of systemic scleroderma as a special form in which another disease with a clear nosological outline is “superimposed” on the manifestations of SJS.
Most often, systemic scleroderma is combined with rheumatoid arthritis and dermatopolymyositis. In 70% of patients with a cross form, signs of two diseases are observed already during the first 3 years of the disease.
The cross-form of systemic scleroderma is characterized by a subacute (52%) and chronic (42%) course, a predominance of a limited type of skin lesions, a rare development of digital ulcers and necrosis, telangiectasias and osteolysis, less severe damage to internal organs . At the same time, this form of systemic scleroderma is characterized by high inflammatory activity with significant immunological disorders.
Share:
Add a comment