The term scleroderma is commonly used to refer to any member of a group of six related disorders. All six are types of autoimmune cutaneous sclerosis that cause damage to the arteries, joints and internal organs. In this paper, I will present an in-depth examination of the current body of scientific knowledge regarding scleroderma, from the epidemiology and pathogenesis of the disease to the newest treatment options researchers are investigating.
According to statistics issued by the Scleroderma Foundation, approximately 150,000 to 500,000 people in the United States alone are classified as having developed one of the variations of the rheumatic disease referred to as scleroderma: linear scleroderma (also known as un coup de sabre), morphea scleroderma, CREST (an acronym referring to a set of symptoms related to systemic scleroderma), limited scleroderma, diffuse scleroderma, and systemic sclerosis sans scleroderma. In order to facilitate understanding, I will refer to all of these conditions using the generic term "scleroderma." I will differentiate between the variations of the disorder only as the subject matter demands. In the general population, the incidence of scleroderma sufferers is about 30 per 100,000. Women appear most frequently in this population; female scleroderma patients outnumber males fifteen-to-one . Among the patients afflicted, most are middle-aged (35 to 54) or older. Scleroderma does not appear to be more likely to afflict members of certain ethnicities over others; however, scientists have observed that the disease behaves more aggressively when seen in non-Caucasian patients. Non-Caucasians presenting with scleroderma tend to "manifest a higher occurrence of critical organ involvement and a worse prognosis".
Although scientists have not yet been able to construct an account of the etiology, cause or mechanism of scleroderma which is universally agreed upon, recent research findings have allowed scientists to make significant advances in the theories attempting to explain the disease's pathogenesis. I will describe some of the most notable scientific speculation in regards to the causation of this disease.
One area researchers are currently looking at closely for clues to the pathogenesis of scleroderma is the autoimmune system. Some scientists believe that the complex hormonal recipe which governs many of the facets of life and health in human females may be in some way responsible for the high prevalence of autoimmune disorders in females, including several conditions related to scleroderma, such as lupus and rheumatoid arthritis.