Does skin disease uniformly predict internal organ involvement?
Not necessarily. Retrospective cohorts and evidence from randomized controlled trials have demonstrated an association between the degree of skin thickening (by mRSS) and subsequent progression of lung disease and overall survival.
In addition, SRC is typically seen in patients with early dcSSc and only rarely encountered in lcSSc.
Nonetheless, an individual patient can manifest signs of internal organ involvement in the absence of skin disease (ssSSc) or can develop progressive internal organ involvement in the setting of established, stable skin disease. In contrast, a patient with severely thickened skin may have little or no internal organ involvement.
Given the potential disconnect between categories of SSc (lcSSc, dcSSc, ssSSc) and internal organ involvement, autoantibody phenotyping and careful assessment for internal organ involvement is required in all patients with SSc.