Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study.

Nathan, SD ; Cottin, V ; et al.

In: The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation, Jg. 40 (2021-06-01), Heft 6, S. 494-503

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Background: Riociguat in Patients with Symptomatic Pulmonary Hypertension associated with Idiopathic Interstitial Pneumonias (RISE-IIP), a randomized, controlled, phase 2b trial of riociguat for pulmonary hypertension associated with idiopathic interstitial pneumonia, was terminated early due to increased mortality in riociguat-treated patients. Baseline characteristics of enrolled patients demonstrated a low diffusing capacity of the lung for carbon monoxide (DL CO ) with preserved lung volumes at baseline, suggesting the presence of combined pulmonary fibrosis and emphysema (CPFE) in some patients. This post hoc analysis of RISE-IIP was undertaken to explore lung morphology, assessed by high-resolution computed tomography, and associated clinical outcomes.

Methods: Available baseline/pre-baseline high-resolution computed tomography scans were reviewed centrally by 2 radiologists. The extent of emphysema and fibrosis was retrospectively scored and combined to provide the total CPFE score.

Results: Data were available for 65/147 patients (44%), including 15/27 fatal cases (56%). Of these, 41/65 patients (63%) had CPFE. Mortality was higher in patients with CPFE (12/41; 29%) than those without (3/24; 13%). Fourteen patients with CPFE had emphysema > fibrosis (4 died). No relationship was observed between CPFE score, survival status, and treatment assignment. A low DL CO , short 6-min walking distance, and high forced vital capacity:DL CO ratio at baseline also appeared to be risk factors for mortality.

Conclusions: High parenchymal lung disease burden and the presence of more emphysema than fibrosis might have predisposed patients with pulmonary hypertension associated with idiopathic interstitial pneumonia to poor outcomes in RISE-IIP. Future studies of therapy for group 3 pulmonary hypertension should include centrally adjudicated imaging for morphologic phenotyping and disease burden evaluation during screening.

Titel:	Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study.
Autor/in / Beteiligte Person:	Nathan, SD ; Cottin, V ; Behr, J ; Hoeper, MM ; Martinez, FJ ; Corte, TJ ; Keogh, AM ; Leuchte, H ; Mogulkoc, N ; Ulrich, S ; Wuyts, WA ; Yao, Z ; Ley-Zaporozhan, J ; Müller-Lisse, UG ; Scholle, FD ; Brüggenwerth, G ; Busse, D ; Nikkho, S ; Wells, AU
Zeitschrift:	The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation, Jg. 40 (2021-06-01), Heft 6, S. 494-503
Veröffentlichung:	1999- : New York, NY : Elsevier ; <i>Original Publication</i>: St. Louis, Mo. : Mosby-Year Book, Inc., c1991-, 2021
Medientyp:	academicJournal
ISSN:	1557-3117 (electronic)
DOI:	10.1016/j.healun.2021.02.006
Schlagwort:	Aged Dose-Response Relationship, Drug Female Forced Expiratory Volume physiology Humans Hypertension, Pulmonary etiology Hypertension, Pulmonary physiopathology Idiopathic Interstitial Pneumonias diagnosis Idiopathic Interstitial Pneumonias physiopathology Lung physiopathology Male Prognosis Pulmonary Wedge Pressure drug effects Retrospective Studies Tomography, X-Ray Computed methods Vital Capacity physiology Hypertension, Pulmonary drug therapy Idiopathic Interstitial Pneumonias complications Lung diagnostic imaging Pulmonary Wedge Pressure physiology Pyrazoles administration & dosage Pyrimidines administration & dosage
Sonstiges:	Nachgewiesen in: MEDLINE Sprachen: English Publication Type: Clinical Trial, Phase II; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't Language: English [J Heart Lung Transplant] 2021 Jun; Vol. 40 (6), pp. 494-503. <i>Date of Electronic Publication: </i>2021 Feb 19. MeSH Terms: Hypertension, Pulmonary / drug therapy ; Idiopathic Interstitial Pneumonias / complications ; Lung / diagnostic imaging ; Pulmonary Wedge Pressure / physiology ; Pyrazoles / administration & dosage ; Pyrimidines / administration & dosage ; Aged ; Dose-Response Relationship, Drug ; Female ; Forced Expiratory Volume / physiology ; Humans ; Hypertension, Pulmonary / etiology ; Hypertension, Pulmonary / physiopathology ; Idiopathic Interstitial Pneumonias / diagnosis ; Idiopathic Interstitial Pneumonias / physiopathology ; Lung / physiopathology ; Male ; Prognosis ; Pulmonary Wedge Pressure / drug effects ; Retrospective Studies ; Tomography, X-Ray Computed / methods ; Vital Capacity / physiology Contributed Indexing: Keywords: Clinical trials registration. NCT02138825; combined pulmonary fibrosis and emphysema; high-resolution computed tomography; pulmonary hypertension associated with idiopathic interstitial pneumonia; riociguat Substance Nomenclature: 0 (Pyrazoles) ; 0 (Pyrimidines) ; RU3FE2Y4XI (riociguat) Entry Date(s): Date Created: 20210321 Date Completed: 20220131 Latest Revision: 20220131 Update Code: 20240513

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