J Respir Med Lung Dis | Volume 1, Issue 1 | Case Report | Open Access

Epidemiology Screening Diagnosis and Treatment of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

Kavita B Khaira^1*, Mark V Avdalovic², Charles K Whitcomb³, Roblee Allen² and Ezra A Amsterdam³

¹Department of Internal Medicine, UC Davis Medical Center, USA
²Department of Pulmonary and Critical Care Medicine, UC Davis Medical Center, USA
³Department of Cardiovascular Medicine, UC Davis Medical Center, USA

^*Correspondance to: Kavita B Khaira 

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Abstract

Systemic sclerosis (SSc) is a very rare rheumatologic disorder with systemic involvement including pulmonary, gastrointestinal, renal and musculoskeletal complications. Pulmonary hypertension is an important manifestation of SSc with multiple etiologies, including pulmonary arterial hypertension (PAH), left heart dysfunction, interstitial lung disease and chronic thromboembolic disease. PAH is present in 7-12% of SSc patients and is a leading cause of death in this population with a 2-year mortality of >60%. Routine screening for pulmonary hypertension is warranted in this group and results in earlier detection and initiation of therapy, which may result in improved outcomes. PAH treatment includes use of prostanoids, endothelin receptor antagonists, and/or phosphodiesterase-5 inhibitors/guanylate cyclase stimulators as monotherapy or in combination. This review will consider the multiple etiologies of pulmonary hypertension in SSc and address the epidemiology, clinical manifestations, screening guidelines, diagnostic considerations, current treatment and prognosis of SSc-associated PAH.

Citation:

Khaira KB, Avdalovic MV, Whitcomb CK, Allen R, Amsterdam EA. Epidemiology Screening Diagnosis and Treatment of Systemic Sclerosis- Associated Pulmonary Arterial Hypertension. J Respir Med Lung Dis. 2016; 1(1): 1003.