Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being
Section snippets
Natural history of disease
Systemic sclerosis (SSc) is a family of diseases with a wide spectrum of clinical and laboratory manifestations [1]. Its natural history is most easily understood by dividing the two major clinical variants into early and late stages and tracking skin thickness and the appearance of other disease features over time (Fig. 1). For this purpose, we have arbitrarily defined SSc with diffuse cutaneous involvement (dcSSc) as skin thickening proximal to the elbows and knees (upper arms, thighs,
Assessment of disease activity and damage
The status of a patient or the severity of his/her disease at a given time represents some combination of irreversible tissue damage and potentially reversible disease activity. This distinction is important. If disease activity is present, it may be followed in the future by irreversible damage, but also may be amenable to some type of intervention before additional damage has occurred. If damage is “end-stage,” which requires artificial support, then the only logical intervention which could
Functional status and psychologic well-being
Clinical information and the results of laboratory tests do not measure the impact of a disease, such as SSc, on a patient's activities of daily living, functional capacity, or emotional well-being. Although few health outcome instruments have been developed and validated specifically for SSc, there are some publications and active ongoing efforts in this area, which were thoroughly reviewed by Merkel [74].
So-called “quality of life” instruments, such as the Sickness Impact Profile [75] and the
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