Clinical presentation of patient with systemic sclerosis

Sistemska skleroza (SSc) bolest je vezivnog tkiva nepoznate etiologije. Osnovne značajke SSc-a su autoimunost, upala, proširena vaskulopatija te intersticijska i perivaskularna fibroza. Klinička prezentacija sistemske skleroze je raznolika. Uz opće simptome, dominantno se prezentira s kožnim i krvožilnim promjenama te promjenama na unutarnjim organima. Raynaudov fenomen, reverzibilni vazospazam distalnih dijelova tijela, prisutan je u više od 90% bolesnika sa SSc-om i nerijetko je prvi, nagovještavajući simptom SSc-a. Raynaudov fenomen zajedno s oštećenjem malih krvnih žila dovodi do razvoja digitalnih ulceracija. U ovom radu uz pregled dosadašnjeg znanja o sistemskoj sklerozi prikazana je i bolesnica s digitalnom ulceracijom kao komplikacijom sistemske skleroze, koja je liječena kombinirano intravenskom primjenom prostaglandina (iloprost) i hiperbaričnom oksigenoterapijom.Systemic sclerosis (SSc) is a connective tissue disease of an unknown etiology. The hallmarks of SSc are autoimmunity, inflammation, widespread vasculopathy and progressive interstitial and perivascular fibrosis. The clinical presentation of systemic sclerosis is diverse. Along with general symptoms, SSc presents predominantly with skin and vascular changes and changes in internal organs. Raynaud's phenomenon, reversible vasospasm of the distal parts of the body, is present in more than 90% of patients with SSc often as the initial manifestation of the disease. Raynaud's phenomenon, along with the damage of small blood vessels leads to the development of digital ulcerations. This paper presents an overview of current knowledge about systemic sclerosis and a patient with digital ulceration as a complication of systemic sclerosis, which is treated with a combination of intravenous administration of prostaglandin (iloprost) and hyperbaric oxygen therapy