Everything you always wanted to know about systemic sclerosis but were afraid to ask: Part 5. Treatment of patients with systemic sclerosis characteristics and treatment recommendations for interstitial lung disease, cardiac involvement, pulmonary arterial hypertension, musculoskeletal, gastrointestinal and renal involvement

Systemic sclerosis (SSc) is a systemic connective tissue disease characterised by disseminated 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 induration of the skin all over her body. The disease is a rare condition, with an estimated 1 in 10 000 people in Poland suffering from SSC. Women predominate among patients, with a 3–4-fold advantage over men. The disease typically has its onset between the ages of 30 and 50. Early detection and treatment of organ complications are key to improving quality of life and reducing mortality in SSc patients. Given the considerable variability of the clinical course, it seems justified to take an individual approach to patients and cooperate with multiple specialists, both at the stage of diagnosis and at the stage of treatment. Treatment is based on what is known as an organ-specific strategy and consists of tailoring pharmacotherapy to the clinical presentation, disease period and organ complications. In addition to pharmacology, the treatment of patients should also include education of the patient and their family, and — if necessary — surgical treatment or other necessary interventions