Introduction to section VIII: Management and outcome assessment

Scleroderma has complex pathogenesis, and its protean clinical and pathological manifestations reflect a disease process affecting multiple organs. In addition, scleroderma encompasses a series of related but distinct entities that are characterized by different course, severity, response to therapy, and outcomes. Scleroderma causes pain, disfigurement, functional impairment, and a substantial decrease in life expectancy. The survival of patients with scleroderma has shown improvement during the past two decades. This improvement in survival, as well as in disease-associated morbidity and disability, is due to advances in diagnosis and early recognition of internal organ involvement, as well as successful management of selected complications such as pulmonary arterial hypertension, Raynaud phenomenon, gastroesophageal reflux disease, ischemic digital ulcers, and scleroderma renal crisis. Additionally, recognition of prognostic risk factors, such as scleroderma-associated autoantibodies, has permitted better risk stratification, which is indispensible for both more effective clinical trials, as well for optimized patient management. Risk stratification in scleroderma enables the selection of more precise therapies targeted to the individual scleroderma patient while reducing potential drug toxicities.