Systemic sclerosis (scleroderma) and related disorders: clinical aspects.

To adequately evaluate and manage patients with systemic sclerosis, practitioners and patients alike must have a working knowledge of the natural history of the disease. What we know generally about the natural history should be coupled with four pieces of patient specific clinical information. (i) Does the patient have systemic sclerosis? (ii) How long has the patient had systemic sclerosis? (iii) How extensive was/is the sclerodermatous skin induration/ thickening at its worst? (iv) Is there presently evidence of visceral involvement (heart, lung, kidney, gastrointestinal, musculoskeletal)? This chapter will show you how to use this information to construct (for you and the patient) a picture of where that patient is presently in the natural history, where the patient is likely to be in the future (in general terms), what visceral involvements are likely or unlikely to occur, what evaluations are needed now and in the future, and what treatments are recommended and why.