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Abstract Number: 1678

Initial Therapy with an Endothelin Receptor Antagonist Is Associated with Worse Outcomes in Patients with Systemic Sclerosis and Pulmonary Arterial Hypertension: Observations from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Cohort

Matthew R. Lammi1, Lesley Ann Saketkoo2, Stephen C. Mathai3, Robyn T. Domsic4, Christine M. Bojanowski5, Virginia D. Steen6, Daniel E. Furst7 and Pharos Investigators8, 1Section of Pulmonary and Critical Care Medicine, LSU Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, 2LSU Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, 3Medicine, Johns Hopkins University, Baltimore, MD, 4Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA, 5Internal Medicine and Pediatrics, LSU Health Sciences Center, New Orleans, LA, 6Department of Rheumatology, Georgetown University Medical Center, Washington, DC, 7University of California, Los Angeles, Department of Medicine, Los Angeles, CA, 8Georgetown University Medical Center, Washington, DC

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: hypertension, pulmonary complications, systemic sclerosis and treatment options

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Session Information

Session Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics: Systemic Sclerosis, Diagnostic and Therapeutic Aspects

Session Type: Abstract Submissions (ACR)

Background/Purpose: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc).  Although medications have improved their prognosis, optimal therapy remains undefined. The goal of this study was to compare time to clinical worsening (TTCW) and survival based on initial oral PAH therapy.

Methods: Using data from the PHAROS registry (a multicenter prospective observational study enrolling SSc patients with incident pulmonary hypertension), patients with group I PAH, 6 months of initial therapy with either an endothelin-receptor antagonist (ERA), phosphodiesterase-5 inhibitor (PDE5), or a combination of ERA/PDE5 were included. Patients treated initially with prostacyclins were excluded. The starting point for all analyses was the date of therapy initiation.  Outcomes were survival and TTCW, defined as the first occurrence of death, PAH-related hospitalization, lung transplant, initiation of parenteral prostacyclin, or worsening symptoms.  

Results:   Ninety-eight patients (initial ERA=24, initial PDE5=59, initial ERA/PDE5=15) were included; no significant differences in baseline variables existed.  TTCW was significantly worse in patients initially started on ERA compared to PDE5 or ERA/PDE5 (p=0.0001, Fig 1).  Baseline factors independently associated with shorter TTCW were initial ERA (HR 0.38, p=0.009), lower DLCO (HR 0.69 per 10% change, p=0.04), and higher PVR (HR 1.10 per Wood unit change, p=0.007).  Three year survival was significantly worse in the initial ERA group (52.9%) compared to the PDE5 (91.5%) or ERA/PDE5 group (92.9%, p=0.004, Fig 2).  The only baseline factor independently associated with risk for death in this cohort was initial ERA therapy (HR 0.22, p=0.004). 

 

Conclusion: Compared to PDE5 or combination ERA/PDE5, initial therapy with an ERA in SSc-PAH patients was associated with a significantly worse TTCW and survival, even after adjustment for commonly accepted prognostic factors. Although these findings may be the result of unmeasured imbalances between groups, it is plausible that known ERA side effects such as fluid retention may have led to clinical worsening. Further study into the optimal initial oral therapy in patients with SSc-PAH is needed.

 



Disclosure:

M. R. Lammi,
None;

L. A. Saketkoo,
None;

S. C. Mathai,
None;

R. T. Domsic,
None;

C. M. Bojanowski,
None;

V. D. Steen,

Actelion Pharmaceuticals US,

8,

United Therapeutics,

5,

Gilead Science,

8,

Roche Pharmaceuticals,

2,

Sanofi-Aventis Pharmaceutical,

2,

CSL Berhing,

2,

Intermune,

2,

Bayer,

5;

D. E. Furst,
None;

P. Investigators,
None.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/initial-therapy-with-an-endothelin-receptor-antagonist-is-associated-with-worse-outcomes-in-patients-with-systemic-sclerosis-and-pulmonary-arterial-hypertension-observations-from-the-pulmonary-hypert/

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