Table 2 Overarching principals

1) Always approach systemic sclerosis as a multi-organ disease

2) Chronological involvement of the skin and organ involvement in both systemic sclerosis (Fig. 1) as well as scleroderma (Fig. 2) is important as it might have therapeutic implications and prophylactic impact

3) Systemic Sclerosis (SSc) has the highest mortality of any of the autoimmune rheumatic diseases, with approximately half of the people affected by SSc eventually dying as a direct result of the disease or a related complication(s)

4) Timely diagnosis of SSc is vital and delays in diagnosis should be minimized

5) Once a confirmed diagnosis is established, all patients can be designated as either lcSSc or dcSSc subset based upon the extent of skin thickening

6) Proximal skin involvement, involving the skin of the trunk or proximal limbs, is designated diffuse

7) Of people with SSc, 1 in 5 develop overlap connective tissue diseases, therefore, cases with overlap disease should be identified so that overlap features may be treated concurrently with SSc

8) All patients require symptomatic treatment, and both limited and diffuse cases should be treated for vascular manifestations

9) Active, early dcSSc requires immunosuppressive treatment

10) In all cases of SSc, watchful follow-up to determine significant organ-based complications is mandatory

11) Patient-reported outcomes can be of help to monitor the individual patient’s disease activity status, the development of organ affection, identify his/her targets, and set up self-management programs

12) Shared decision-making should be the base for all management strategies offered to the patient