Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease.

Publisher: Wiley Country of Publication: United States NLM ID: 101623795 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2326-5205 (Electronic) Linking ISSN: 23265191 NLM ISO Abbreviation: Arthritis Rheumatol Subsets: MEDLINE

Original Publication: Malden, MA : Wiley, [2014]-

Hemodynamics*; Endothelin Receptor Antagonists/*therapeutic use ; Immunosuppressive Agents/*therapeutic use ; Lung Diseases, Interstitial/*drug therapy ; Phosphodiesterase 5 Inhibitors/*therapeutic use ; Pulmonary Arterial Hypertension/*drug therapy ; Scleroderma, Systemic/*drug therapy; Aged ; Cardiac Catheterization ; Epoprostenol/analogs & derivatives ; Female ; Forced Expiratory Volume ; Humans ; Hypertension, Pulmonary/complications ; Hypertension, Pulmonary/drug therapy ; Hypertension, Pulmonary/physiopathology ; Lung Diseases, Interstitial/complications ; Lung Diseases, Interstitial/physiopathology ; Male ; Middle Aged ; Oxygen Inhalation Therapy/statistics & numerical data ; Prognosis ; Pulmonary Arterial Hypertension/complications ; Pulmonary Arterial Hypertension/physiopathology ; Pulmonary Diffusing Capacity ; Scleroderma, Systemic/complications ; Scleroderma, Systemic/physiopathology ; Survival Rate ; Treatment Outcome ; Vital Capacity ; Walk Test

Objective: Patients with systemic sclerosis and both pulmonary hypertension and interstitial lung disease (SSc-PH-ILD) generally carry a worse prognosis than patients with SSc and pulmonary arterial hypertension (SSc-PAH) without ILD. There is no evidence of the efficacy of PAH therapies in SSc-PH-ILD. We undertook this study to compare survival of and response to treatment in patients with SSc-PH-ILD and those with SSc-PAH.
Methods: We analyzed 128 patients (66 with SSc-PH-ILD and 62 with SSc-PAH) from 15 centers, in whom PH was diagnosed by right-sided heart catheterization; they were prospectively included in the PH registry. All patients received PAH-specific therapy. Computed tomography of the chest was used to confirm or exclude ILD.
Results: At baseline, patients with SSc-PH-ILD had less severe hemodynamic impairment than those with SSc-PAH (pulmonary vascular resistance 5.7 Wood units versus 8.7 Wood units; P = 0.0005) and lower diffusing capacity for carbon monoxide (median 25% [interquartile range (IQR) 18%, 35%] versus 40% [IQR 31%, 51%]; P = 0.0005). Additionally, patients with SSc-PH-ILD had increased mortality (8.1% at 1 year, 21.2% at 2 years, and 41.5% at 3 years) compared to those with SSc-PAH (4.1%, 8.7%, and 21.4%, respectively; P = 0.04). Upon treatment with PAH-targeted therapy, no improvement in the 6-minute walk distance was observed in either group. Improvement in the World Health Organization functional class was observed less frequently in patients with SSc-ILD-PH compared to those with SSc-PAH (13.6% versus 33.3%; P = 0.02). Hemodynamics improved similarly in both groups.
Conclusion: ILD confers a worse prognosis to SSc-PH. Response to PAH-specific therapy is clinically poor in SSc-PH-ILD but was not found to be hemodynamically different from the response observed in SSc-PAH.
(© 2020, American College of Rheumatology.)

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0 (Endothelin Receptor Antagonists)
0 (Immunosuppressive Agents)
0 (Phosphodiesterase 5 Inhibitors)
DCR9Z582X0 (Epoprostenol)

Date Created: 20200906 Date Completed: 20210301 Latest Revision: 20210301

20250114

10.1002/art.41512

32892515