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[Systemic sclerosis in childhood].

F Falcini¹, S Trapani, G Taccetti

¹Dipartimento di Pediatria, Università degli Studi, Firenze, Italia.

La Pediatria Medica E Chirurgica : Medical and Surgical Pediatrics

|November 1, 1990

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Systemic sclerosis (SS) is a complex condition causing inflammation and fibrosis affecting skin and internal organs. Treatment focuses on improving circulation and reducing collagen buildup to manage symptoms.

Area of Science:

Rheumatology
Immunology
Dermatology

Background:

Systemic sclerosis (SS) is a multisystem autoimmune disease of unknown etiology.
Characterized by inflammation, vascular damage, and excessive connective tissue accumulation.
Lesions affect skin and internal organs like kidneys, lungs, heart, and gastrointestinal tract.

Purpose of the Study:

To provide an overview of systemic sclerosis.
To describe its characteristic pathological changes and clinical manifestations.
To outline current treatment strategies.

Main Methods:

Literature review of systemic sclerosis.
Analysis of pathological and clinical features.
Summary of therapeutic approaches.

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Main Results:

Early symptoms include Raynaud's phenomenon followed by skin changes (edema, thickening, atrophy).
Prognosis is significantly influenced by the extent of visceral organ involvement.
Treatment involves medications to enhance microcirculation and inhibit collagen proliferation.

Conclusions:

Systemic sclerosis is a progressive fibrotic disease with diverse organ involvement.
Effective management requires addressing microcirculatory dysfunction and fibrotic processes.
Pharmacological interventions like calcium-channel blockers and D-Penicillamine are key treatment components.