Background: In Systemic sclerosis (SSc) reduced capillary density decreases blood flow and leads to tissue ischemia and fingertip ulcers. Nailfold videocapillaroscopy (NVC), a diagnostic and follow-up parameter, useful to evaluate the severity, activity and the stage of SSc microvascular damage. Autologous Hemopoietic Stem Cells Transplantation (HSCT) is a new treatment for severe diffuse cutaneous systemic sclerosis (dcSSc) patients refractory to conventional therapies.
Objectives: to evaluate the improvement of microvasculature after HSCT.
Methods and results: 16 severe dcSSc patients, with a “late” videocapillaroscopy pattern underwent an immunesuppressive treatment: 6 were treated with HSCT and 10 with monthly pulse cyclophosphamide (CYC) 1 g for 6 months and then orally with 50 mg/day for further six months. NVC was performed before and after 3 months from the beginning of each treatment and then repeated every 3 months. In all patients, before HSCT, NVC large avascular areas and ramified capillaries and vascular architectural disorganization (“late” pattern). Three months after HSCT, NVC pattern changed from “late” into “active”, showing frequent giant capillaries (>6/mm) and haemorrhages, absence of avascular areas and angiogenesis phenomena; after 1 year from HSCT, microvascular abnormalities were still in the “active” pattern. In patients treated with CYC, no NVC modifications were observed during 24 months of follow up and the pattern remained always “late”.
Conclusion: These results indicate that HSCT with high dose CYC regimen may foster vascular remodelling while CYC at lower doses and with a chronic regimen does not influence the microvasculature.