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Systemic sclerosis (SSc) is a multisystem autoimmune disease with the highest case-specific mortality of the autoimmune illness. The most common causes of SSc-related death are pulmonary hypertension and interstitial lung disease (ILD). In total, these two complications account for over half of SSc-related deaths. It has been well recognized that pulmonary arterial hypertension (PAH) in SSc patients portends a worse prognosis than PAH from other causes. Although survival in SSc-related PAH has improved with modern therapeutics, as we will discuss below, SSc-PAH survival still lags behind that of survival with PAH from other causes. It has been shown that systematic pulmonary hypertension (PH) screening programs in SSc are associated with better survival, likely due to earlier detection and intervention. This highlights the need for rheumatologists to maintain a high clinical suspicion for pulmonary hypertension and appropriately screen their SSc patients for the presence of this complication.
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Dr. Domsic has financial interests with the following any entity or entities producing health care goods or services as indicated below:
Dr. Risbano has financial interests with the following any entity or entities producing health care goods or services as indicated below:
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Release Date: 3/7/2017 | Last Modified On: 3/7/2017 | Expires: 3/7/2018