Early and rapidly progressive systemic sclerosis with skin or internal organ involvement, predominantly of the lung, is associated with 3–5-year survival of 50–80%. In the past decade, despite improved screening for systemic sclerosis and new drugs for pulmonary hypertension, no treatment has been effective for the 10–20% of patients with life-threatening disease. After the first use of haemopoietic stem-cell transplantation (HSCT) for systemic sclerosis in 1997, several phase 1 and 2 studies and lung imaging.