systemic sclerosis

Summary

Background. One of the main aspects in systemic sclerosis pathogenesis is small vessel vasculopathy. Capillary damage adversely affects the quality of life by causing pain, impairment of hand function and ulceration of the fingertips, which can lead to gangrene, amputations and disability. Autologous haematopoietic stem cell transplantation modifies fibrosis, activation of the autoimmune system and stabilises the function of the internal organs, but its effect on microcirculation and capillary regeneration is still largely unknown.

Summary

Background. Systemic sclerosis is a rare autoimmune connective tissue disease that affects the skin and many internal organs and is associated with impaired qual ity of life and the risk of premature death. Autologous hematopoietic stem cell transplantation in hibits disease progression and significantly improves the clinical condition of the patients, but can lead to many serious complications.

Systemic sclerosis is a rare multisystem connective tissue disease characterized by a vasculopathy, diffuse fibrosis and immune abnormalities. This disease has a high mortality rate, especially if it presents with concomitant disorders, such as interstitial lung disease or pulmonary arterial hypertension. Although pulmonary arterial hypertension is a rare disease, with an estimated frequency of 15-50 cases per million, the prevalence of this condition in patients with systemic sclerosis has been reported to be 7-12%.

Relevant animal models are essential tools to investigate in depth the patho-genesis of systemic sclerosis and search of new therapeutic interventions. Several models of scleroderma are currently available but, unfortunately, none of these reflects all features of the human disease. Some of these models present inflammation followed by fibro-sis, whether some others primarily mimic autonomous fibroblast activation. Only few models are available and suitable to study small vessels vasculo-pathy. Hence, we provide an overview of the most import ant models of systemic sclerosis.