Sindrome “overlap” sclerosi sistemica/sindrome da anticorpi antifosfolipidi: descrizione di un caso complesso per la gestione multispecialistica e per le strategie terapeutiche adottate

Systemic sclerosis (SSc) is a complex autoimmune disease. Typical features of the disease include thickening of the skin (scleroderma) and damage to other organs, caused by excessive accumulation of collagen. Vascular disease is universal in patients with SSc, but there is a wide variability in its severity. It is clear that an early insult to the microvasculature is followed by an on-going chronic process. This results in profound vascular damage in a subset of patients who develop severe events such as digital loss and pulmonary arterial hypertension. In addition, the presence of antiphospholipid antibodies have been associated with a variety of clinical manifestations related to thrombotic events. Both SSc and antiphospholipid syndrome cause small vessel vasculopathy and it’s reasonable to think that both diseases can be synergic in inducing ischemic features. The aim of this article is to describe, through presentation of a case report, vascular manifestations of SSc with antiphospolipid antibody positivity and their serious consequences on peripheric circulation. We describe the case of a woman suffering from overlap syndrome (systemic sclerosis/antiphospholipid syndrome) with ulcers due both to scleroderma vasculopathy and thrombophilic syndrome. She was treated with conventional immunosuppressive drugs, i.v. immunoglobulins, and plasmapheresis in association with vascular therapies. The failure of all medical therapies led us toward an experimental attempt with mesenchymal stem cells.