…that secondary pulmonary complications are the leading cause of disease-related morbidity and mortality in patients with scleroderma?
Scleroderma, also known as systemic sclerosis, is a rare, chronic, connective tissue disorder that typically affects women between the ages of 30 and 60. Though the term scleroderma (from the Greek for "hard skin") was coined by Giovambattista Fantonetti in the 1830s, the disease has far earlier origins. The initial description of a scleroderma patient may have been made by Hippocrates when he described a man with general pruritus and thick skin in the early 4th century B.C.E . The first generally accepted modern description of a patient with scleroderma was published by Carlo Curzio in 1753 . Despite this long and varied history, surprisingly little is known about either the underlying causes or the factors that regulate disease progression.
As scleroderma progresses it impacts many other organ systems. Most scleroderma patients exhibit some level of pulmonary injury. The pulmonary manifestations will typically comprise either fibrosis or pulmonary arterial hypertension (PAH) . While the underlying trigger that instigates pulmonary vascular damage in scleroderma is unknown, it is likely to involve a cascade of inflammatory and fibrotic processes that leads to a stiffening of the pulmonary vessels and airways . The associated lung disease accounts for roughly one third of all deaths in scleroderma patients . Though the median 10-year survival for scleroderma patients is 65%, patients who develop PAH have an average survival of less than two years .
PAH can be defined simply as the increase of the mean pulmonary artery pressure from the normal 12-16 mm Hg to greater than 25 mm Hg. PAH can also be associated with numerous other diseases, involve both genetic and environmental components, and comprise a range of pathological symptoms . PAH is a progressive disorder that can lead to difficulty breathing, diminished activity levels, vascular remodeling, right ventricular dysfunction, and eventually heart failure and death.
Currently, the only "curative" therapy available for PAH is lung transplantation. However, lung transplantation is extremely rare in scleroderma patients. The standard methods of treatment for PAH are directed toward improving quality of life, delaying clinical deterioration, and improving mean survival time. Presently, the most important factor is early diagnosis of pulmonary disease, which allows for intervention of the pulmonary remodeling process and significantly improves the prognosis. Currently approved treatments for PAH in scleroderma patients include prostacyclins, endothelin-receptor agonists, and phosphodiesterase inhibitors. Supplemental oxygen is also used to improve arterial oxygenation and patient comfort. Pulmonary complications remain the primary determinate of morbidity and mortality in scleroderma patients, research conducted in the last decade could soon provide a more effective treatment for this debilitating disease.
Author: David Clark
Chief Editor: Natalie N. Bauer, Ph.D., Dec. 2009
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