Consequences of PAH-CTD

Decreased Survival

In the REVEAL Registry, patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) had significantly worse survival than patients with idiopathic PAH (IPAH; P=0.017).1

Decreased survival in PAH-CTD1
Decreased survival chart for PAH-CTD Decreased survival chart for PAH-CTD
Reprinted from CHEST, Vol 142, Benza RL, et al, An Evaluation of Long-term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From the REVEAL Registry, pp448-456, 2012, with permission from Elsevier.

Among patients in the PHAROS Registry with PAH associated with systemic sclerosis (SSc), or scleroderma, PAH WAS THE LEADING CAUSE OF DEATH (median follow-up time, 7.1 years).2

PAH-CTD causes of death pie chart PAH-CTD causes of death pie chart
Shared pathophysiologic features between SSc and PAH may contribute to advanced presentation and poor prognosis at diagnosis.3-7 See the PAH-SSc Link >

Rapid Progression

PAH-SSc can progress quickly, drastically shortening the lives of patients:

Human figure silhouettes half-filled in

In the REVEAL Registry,

49%

of patients with newly diagnosed PAH-SSc died within 3 years of PAH diagnosis.8‡

Use minimally invasive tests to screen for possible PAH-SSc and the need for right heart catheterization.9 Learn More >
*P value vs IPAH.
The PHAROS Registry was partially funded by Actelion Pharmaceuticals Ltd. Percentages do not add up to 100% due to rounding.
In the REVEAL Registry, patients with new diagnoses were defined as those enrolled within 90 days of diagnostic right-sided heart catheterization.8 REVEAL was funded and sponsored by Actelion Pharmaceuticals US, Inc.
PAH-SSc=pulmonary arterial hypertension associated with systemic sclerosis; PHAROS=Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma; REVEAL=Registry to EValuate EArly and Long-term PAH Disease Management.
References: 1. Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. CHEST. 2012;142(2):448-456. 2. Kolstad KD, Li S, Steen V, Chung L; PHAROS Investigators. Long-term outcomes in systemic sclerosis-associated pulmonary arterial hypertension from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS). CHEST. 2018;154(4):862-871. 3. Zanatta E, Polito P, Famoso G, et al. Pulmonary arterial hypertension in connective tissue disorders: pathophysiology and treatment. Exp Biol Med. 2019;244(2):120-131. 4. Chaisson NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. CHEST. 2013;144(4):1346-1356. 5. Gaine S. Pulmonary hypertension. JAMA. 2000;284(24):3160-3168. 6. Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease–associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179(2):151-157. 7. Vachiéry J-L, Coghlan G. Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev. 2009;18(113):162-169. 8. Chung L, Farber HW, Benza R, et al. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry. CHEST. 2014;146(6):1494-1504. 9. Khanna D, Gladue H, Channick R, et al. Recommendations for screening and detection of connective-tissue disease associated pulmonary arterial hypertension. Arthritis Rheum. 2013;65(12):3194-3201.