Systémová sklerodermie v roce 2017

Abstract

Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension. The treatment of vascular manifestations uses medicines with vasodilation effect. The manifestation of inflammation is accentuated by pleurisy, pericarditis, myositis, synovitis/arthritis and alveolitis. Finally, the manifestation of fibrosis predominates in association with dermatosclerosis, interstitial lung disease and fibrotic impairment of the gastrointestinal tract. Medicines with immunomodulatory or immunosupressive effects are used to affect the inflammation and fibrosis. Despite the aforementioned, there is still no universally effective treatment available. The pharmacological therapy of this disease is organ specific and symptomatic.Key words: capillaroscopy - digital ulcers - interstitial lung disease - pulmonary arterial hypertension - scleroderma renal crisis - systemic sclerosis.