The first slide is just to introduce myself.
I am Gabriele Valentini and work
at the University Degli Studi della Campania Luigi Vanvitelli,
where I am professor of Rheumatology and the Chief of the Rheumatology Unit.
The next slide shows the nosography of systemic sclerosis.
It is a multi-system
autoimmune rheumatic disease that involves the skin and distinct internal organs.
From a morphological point of view,
it is characterized by three main features: interstitial fibrosis;
microangiopathy, that can be obliterative or dilatative or both;
and proliferative small artery disease.
The next slide depicts the prevalences of
each organ system involving systemic sclerosis.
All the patients present with vascular disease.
The main manifestation of which is represented by Raynaud's phenomenon.
Ninety-three percent of them present with skin sclerosis;
90 percent with the gastrointestinal system manifestations -
mainly esophageal involvement;
70 percent [present] with lung disease -
either interstitial lung disease or pulmonary arterial hypertension;
50 percent with heart disease;
40 percent with the locomotor system disease;
and from three to 15 percent and a median of nine percent, scleroderma renal crisis.