Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

“…However, the variable prevalence of ssSSc among the main studies of the literature might be explained by the use of different classification criteria 8–14. It is supposable that these differences are real and may reflect the variable contribution of genetic and/or geographical/environmental factors among SSc populations from different ethnic groups or geographical areas 21 22. Furthermore, the low rate of ssSSc observed in some reports,9 12 including the present study, could also be related to an inadequate network of specialised tertiary referral centres of some geographic areas where a number of ssSSc may be diagnosed very late or completely overlooked 22…”

“… 21 22 Furthermore, the low rate of ssSSc observed in some reports, 9 12 including the present study, could also be related to an inadequate network of specialised tertiary referral centres of some geographic areas where a number of ssSSc may be diagnosed very late or completely overlooked. 22 …”

Practice pattern for the use of intravenous iloprost for the treatment of peripheral vasculopathy in systemic sclerosis: A case–control study from the Italian national multicenter “SPRING” (Systemic Sclerosis Progression InvestiGation) Registry

“…At the same time, this type of data collection may have some limitations, including the heterogeneity of the involved centers from different areas of the country with potential geographical referral bias. 18 In conclusion, the observed data indicate that the choice of the IV ILO dosage and duration of a single infusion are generally made according to the main recommendations suggested in the datasheet. In particular, the following regimens have been most frequently detected in the Italian centers: Overall, the frequency and dosage of IV ILO administra tion depend on the severity of both peripheral vascular involvement (i.e.…”

“…5 Indeed, our analysis showed that patients from Central Italy more frequently have some features indicating a more severe form of disease, especially regarding peripheral vascular microangiopathy (pitting scars, scleroderma late pattern) similar to what was found in a previous clinicaldemographical analysis of SPRING Registry that has shown as patients from Southern Italy were characterized by a more aggressive disease, account ing for a greater need of IV ILO treatment. 18 The different geographical distribution of SSc subsets has been previ ously emphasized, and may probably be related to referral bias as well to different environmental and/or genetic factors. 18 According to the 2017 EULAR recommendations for the treatment of SSc, IV ILO is indicated for RP manage ment after failure of oral vascular therapies such as CCB and PDE5i or as first choice for DU healing.…”

“…18 The different geographical distribution of SSc subsets has been previ ously emphasized, and may probably be related to referral bias as well to different environmental and/or genetic factors. 18 According to the 2017 EULAR recommendations for the treatment of SSc, IV ILO is indicated for RP manage ment after failure of oral vascular therapies such as CCB and PDE5i or as first choice for DU healing. 6 Almost all cases (99%) complained about RP, while only onethird presented DUs.…”