Juvenile Systemic Scleroderma

Juvenile systemic sclerosis (a synonym for progressive systemic sclerosis) is a chronic polysystemic disease from a group of systemic connective tissue diseases that develops at the age of 16 years and is characterized by progressive fibrotic sclerotic changes of the skin, musculoskeletal system, internal organs and vasospastic reactions like Raynaud's syndrome .

ICD-10 codes

• M32.2. Systemic sclerosis caused by drugs and chemicals.
• M34. Systemic sclerosis.
• M34.0. Progressive systemic sclerosis.
• M34.1. Syndrome CREST.
• M34.8. Other forms of systemic sclerosis.
• M34.9. Systemic sclerosis, unspecified.

Epidemiology of juvenile systemic scleroderma

Juvenile system scleroderma is a rare disease. The primary incidence of juvenile systemic scleroderma is 0.05 per 100 000 population. The prevalence of systemic scleroderma in adults varies between 19-75 cases per 100 000 population, the incidence is 0.45-1.4 per 100 000 population per year, while the proportion of children under 16 years of age among patients with systemic scleroderma is less than 3% , and children under 10 years of age - less than 2%.

Systemic scleroderma in children often begins in preschool and junior school age. At the age of up to 8 years, systemic scleroderma is equally common in boys and girls, and among older children, girls predominate (3: 1).

[1], [2], [3], [4]

Etiology and pathogenesis of juvenile systemic scleroderma

The etiology of scleroderma has not been adequately studied. They assume a complex combination of hypothetical and already known factors: genetic, infectious, chemical, including medicinal, which lead to the launch of a complex of autoimmune and fibro-forming processes, microcirculatory disorders.

Causes of juvenile systemic scleroderma

[5], [6], [7], [8]

Symptoms of juvenile systemic scleroderma

Juvenile systemic scleroderma has a number of features:

• Skin syndrome is often represented by atypical variants (focal or linear lesions, hemiforms);
• the defeat of internal organs and Raynaud's syndrome are less common than in adults, are less clinically pronounced;
• specific for systemic scleroderma immunological markers (antitopoizomeraznye antibodies - Scl-70, and anti-centromere antibodies) are detected less often.

Symptoms of juvenile systemic scleroderma

Classification of juvenile systemic scleroderma

Juvenile systemic scleroderma, in which, apart from the widespread skin lesion, internal organs are involved in the process, it should be distinguished from juvenile and limited scleroderma, which is characterized by the development of sclerosis of the skin and underlying tissues without affecting the vessels and internal organs, although these diseases are often combined with the common term "juvenile scleroderma" .

There is no classification of juvenile systemic scleroderma, therefore a classification developed for adult patients is used.

[9], [10]

Clinical forms of systemic scleroderma

• Prescleroderma. It can be diagnosed in a child with Raynaud's syndrome in the presence of specific antibodies - AT Scl-70, antibodies to the centromere (in the following, in most cases, juvenile systemic scleroderma develops).
• Systemic scleroderma with diffuse skin lesions (diffuse form) - rapidly progressive widespread skin lesions of the proximal and distal parts of the limbs, face, trunk and early defeat (within the first year) of internal organs, detection of antibodies to topoisomerase I (Scl-70).
• Systemic scleroderma with limited skin lesion (acrosclerotic form) is a long isolated Raynaud's syndrome, which precedes limited skin lesions of the distal forearms and hands, legs and feet, late visceral changes, detection of antibodies to the centromere. The peculiarity of juvenile systemic scleroderma is an atypical skin syndrome in the form of focal or linear (on hemitip) skin lesions, which is not a classical acrosclerotic variant.
• Scleroderma without scleroderma - visceral forms, in which the clinical picture is dominated by the defeat of internal organs and Reynaud's syndrome, and skin changes are minimal or absent.
• Cross forms are a combination of signs of systemic scleroderma and other systemic diseases of connective tissue or juvenile rheumatoid arthritis.

The course of systemic scleroderma is acute, subacute and chronic.

Degrees of disease activity: I - minimal, II - moderate and III - maximum. Determination of the degree of activity of systemic scleroderma is conditional and is based on clinical data - the severity of clinical symptoms, the prevalence of the lesion and the rate of progression of the disease.

Stages of systemic scleroderma:

• I - initial, identify 1-3 localization of the disease;
• II - generalization, reflects the systemic, polysyndromic nature of the disease;
• III - late (terminal), there is a failure of the function of one or more organs.

[11], [12], [13], [14], [15], [16]

Diagnosis of juvenile systemic scleroderma

For diagnosis, preliminary diagnostic criteria for juvenile systemic scleroderma developed by European rheumatologists (Pediatric Rheumatology European Society, 2004) are proposed . To establish the diagnosis, 2 large and at least one small criterion are needed.

Diagnosis of juvenile systemic scleroderma

[17], [18]

Treatment of juvenile systemic scleroderma

The patients are shown physiotherapy, massage and physiotherapy exercises, which help maintain the functional capabilities of the musculoskeletal system, strengthen the muscles, expand the amplitude of movements in the joints, and prevent the growth of flexion contractures.

How is Juvenile Systemic Scleroderma Treated?

Prevention of juvenile systemic scleroderma

Primary prevention of juvenile systemic scleroderma is not developed. Secondary prevention consists in preventing relapses of the disease and involves preventing excessive insolation and supercooling of the body, prohibiting the skin from contacting the patient with various chemical reagents and dyes, protecting the skin from possible injuries, unreasonable injections. It is recommended to wear warm clothing, especially gloves and socks, whenever possible to avoid stressful situations, exposure to vibration, smoking, drinking coffee, as well as taking medications that cause vasospasm or increase the viscosity of the blood. Do not take preventive vaccinations in the active period of the disease.

Forecast

The prognosis for life in children with systemic scleroderma is much more favorable than in adults. Mortality in children with systemic scleroderma under the age of 14 years is only 0.04 per 1000 000 population per year. The five-year survival rate of children with systemic scleroderma is 95%. Causes of death - progressive cardiopulmonary insufficiency, scleroderma renal crisis. Possible the formation of pronounced cosmetic defects, disability of patients in connection with the violation of the function of the musculoskeletal system and the development of visceral lesions.

[19], [20], [21], [22], [23], [24], [25]