This varies widely depending on the antibody-based disease variant. Patients with limited systemic scleroderma variants often lead relatively normal lifespans but with steadily increasing disability over time. Patients with diffuse variants have significantly reduced lifespans. Typical 10 year survival rates with diffuse variants is in the 60% range.
The most common causes of death in scleroderma patients is lung involvement followed by heart involvement. Kidney failure is less of an issue than it used to be because of the discovery that ACE inhibitors can be very effective in dealing with scleroderma renal crisis (SRC).
Recent research on autologous stem cell transplants (HSCT), a treatment where the immune system is first destroyed and then rebuilt from the patient's own stem cells, suggests that this may be more effective than conventional treatments in patients with rapidly progressing diffuse disease. However, even with optimum patient selection, there is about a 5% mortality rate for the procedure, which is why it is only being tried in patients with poor prognosis.
Recent research on therapeutic plasma exchange suggests that this may be an effective option but more research is needed to verify this.