UPMC Physician Resources
Pulm/Rheum Grand Rounds Spring 2017: Pulmonary Hypertension in Systemic Sclerosis
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.
Upon completion of this activity, participants should be able to:
- Distinguish the three different WHO Group clinical classifications of pulmonary hypertension that may affect patients with systemic sclerosis.
- Identify systemic sclerosis risk factors for developing WHO Group 1 pulmonary arterial hypertension.
- Identify the appropriate definition of pulmonary arterial hypertension and pharmacologic therapy for patients with systemic sclerosis and pulmonary arterial hypertension.
- Fischer A, Bull TM, Steen VD. Practical approach to screening for scleroderma-associated pulmonary arterial hypertension. Arthritis Care Res (Hoboken). 2012 Mar;64(3):303-10.
- Chaisson, NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. Chest. 2013; 144: 1346-1356.
- Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D42-50.
- Coghlan JG, Galie N, Barbera JA, et al. Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial. Ann Rheum Dis 2016.
Dr. Domsic has financial interests with the following proprietary entity or entities producing health care goods or services as indicated below:
- Grant/Research Support: Bayer Healthcare, Biogen-Idec
- Consultant: Arena Pharmaceuticals
Dr. Risbano has financial interests with the following proprietary entity or entities producing health care goods or services as indicated below:
- Grant/Research Support: Gilead Sciences
- Consultant: Gilead and Aetilion Pharmaceuticals
The University of Pittsburgh School of Medicine is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.
The University of Pittsburgh School of Medicine designates this enduring material for a maximum of .5 AMA PRA Category 1 Credits™. Each physician should only claim credit commensurate with the extent of their participation in the activity. Other health care professionals are awarded (0.05) continuing education units (CEU) which are equivalent to .5 contact hour.
For your credit transcript, please access our website 4 weeks post-completion at http://ccehs.upmc.edu and follow the link to the Credit Transcript page. If you do not provide the last 5 digits of your SSN on the next page you will not be able to access a CME credit transcript. Providing your SSN is voluntary.
Release Date: 3/7/2017 | Last Modified On: 3/7/2017 | Expires: 3/7/2018