Overview: Pathogenesis Integrated

Abstract

The pathogenesis of scleroderma is complex, multifactorial and incompletely understood. Evidence of vascular injury, immune dysfunction and tissue remodeling can be detected in every patient with scleroderma. These processes do not occur in isolation but are interrelated and reciprocally modulate each other, as illustrated in Fig. 14.1 and discussed in the following chapters. The triad of vasculopathy, autoimmunity/inflammation, and connective tissue remodeling underlies the protean clinical and laboratory manifestations of scleroderma, ranging from Raynaud’s phenomenon to autoantibody production to pulmonary fibrosis. However, the relative contributions of these distinct pathophysiological processes to the individual disease phenotype, and their roles in driving the natural history of disease vary greatly from one patient to another.