Scleroderma Subsetting

Abstract

Patients with Systemic Sclerosis [SSc] present great variability in the extent of skin sclerosis, internal organ involvement and prognosis. These aspects have long prompted clinical investigators to differentiate SSc into subsets. The most widely used subsetting schema was proposed by Carwile LeRoy et al. in 1988. The schema differentiates two main subgroups (i.e., limited cutaneous SSc -lcSSc-and diffuse cutaneous SSc -dcSSc-) characterized by different extent of skin sclerosis, autoantibody profile and pattern of internal organ involvement. This schema, however, is based on a Delphy technique (i.e. a consensus among experts). The other available schema, which differentiates three subsets according to skin sclerosis (i.e. lc-SSc characterized by sclerodactyly alone, intermediate cutaneous SSc-ic-SSc where the limbs are also involved, and dc-SSc with trunk skin sclerosis) has been demonstrated to have a greater prognostic power, but lacks any reference to other prognostically relevant items . A prospective multicenter study devoted to improve the three subsetting schema by adding such items could help the clinical investigator to better differentiate SSc subsets.

What does the clinician need to improve patient care in systemic sclerosis?

“…9 11 As pathological alterations and clinical manifestations of SSc are a continuum, any subsetting model involving only the extent of skin changes seems arbitrary and restrictive. 12 Therefore, subsetting classification criteria that include the disease-specific autoantibodies and additional imaging such as nailfold capillaroscopy, validated in a cohort of patients with scleroderma and also with early disease, may aid the development of an optimal classification model.…”