Vol 9, No 3 (2023)
Review paper
Published online: 2023-07-05
Everything you always wanted to know about systemic sclerosis but were afraid to ask: Part 4. Treatment of patients with systemic sclerosis characteristics and recommendations concerning treatment of skin involvement, Raynaud’s phenomenon, calcinosis
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Rheumatology Forum 2023;9(3):125-139.
Abstract
Systemic sclerosis (SSc) is a systemic connective tissue disease marked by diffuse microangiopathy and excessively immune-stimulated fibroblast activity, leading to fibrosis of the skin and internal organs. In the literature, the first report of the disease dates back to 1753 and is attributed to the physician Carlo Curzio of Naples, who described the case of a 17-year-old girl who developed sclerosis of the skin all over her body. The disease is a rare condition. It is estimated that 1 in 10 000 people in Poland suffer from SSc. Women predominate among the patients, with a 3–4-fold prevalence compared to men. Typically, the disease has its onset between 30 and 50 years of age. Early detection and treatment of organ complications are key to improving quality of life and reducing mortality in patients with SSc. Given the significant variability in the clinical course, an individualised approach to patients and multidisciplinary collaboration appear to be justified, both in the diagnostic and treatment phases. The treatment is based on the organ-specific therapeutic strategy, which involves tailoring the pharmacotherapy to the clinical presentation, disease stage, and organ complications. Treatment of patients should include, in addition to pharmacology, education of the patient and family and, if necessary, surgical treatment or other necessary interventions.
Keywords: systemic sclerosisrecommendationstreatmentmanagementrituximabnintedanibtocilizumab
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