open access

Vol 85, No 4 (2017)
REVIEWS
Published online: 2017-08-28
Submitted: 2017-04-28
Accepted: 2017-08-10
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Pulmonary hypertension in diffuse parenchymal lung diseases — is there any benefit of PAH-specific therapy?

Monika Szturmowicz, Aneta Kacprzak, Jan Kuś
DOI: 10.5603/ARM.2017.0036
·
Adv Respir Med 2017;85(4):216-223.

open access

Vol 85, No 4 (2017)
REVIEWS
Published online: 2017-08-28
Submitted: 2017-04-28
Accepted: 2017-08-10

Abstract

Pulmonary hypertension (PH) is diagnosed in 40–50% of the patients with end-stage diffuse parenchymal lung diseases (DPLD), and it is associated with significant worsening of life expectancy. Latest ERS/ESC guidelines recommend best available treatment of DPLD, and long-term oxygen therapy in the patients with PaO2 less than 60 mm Hg. Pulmonary arterial hypertension (PAH)-targeted drugs are not recommended in PH-DPLD patients, due to the risk of increasing the ventilation-perfusion mismatch, and consequently worsening of hypoxaemia. Nevertheless, PAH-oriented treatment may be beneficial to selected groups of patients. The authors try to find the answer to several important questions: is there any benefit of PAH-specific therapy in PH-DPLD, who should be the candidate for PAH-specific therapy, what class of drugs is most promising, and what outcome measures should be employed?

Abstract

Pulmonary hypertension (PH) is diagnosed in 40–50% of the patients with end-stage diffuse parenchymal lung diseases (DPLD), and it is associated with significant worsening of life expectancy. Latest ERS/ESC guidelines recommend best available treatment of DPLD, and long-term oxygen therapy in the patients with PaO2 less than 60 mm Hg. Pulmonary arterial hypertension (PAH)-targeted drugs are not recommended in PH-DPLD patients, due to the risk of increasing the ventilation-perfusion mismatch, and consequently worsening of hypoxaemia. Nevertheless, PAH-oriented treatment may be beneficial to selected groups of patients. The authors try to find the answer to several important questions: is there any benefit of PAH-specific therapy in PH-DPLD, who should be the candidate for PAH-specific therapy, what class of drugs is most promising, and what outcome measures should be employed?

Get Citation

Keywords

pulmonary hypertension, diffuse parenchymal lung diseases, PAH-specific therapy

About this article
Title

Pulmonary hypertension in diffuse parenchymal lung diseases — is there any benefit of PAH-specific therapy?

Journal

Advances in Respiratory Medicine

Issue

Vol 85, No 4 (2017)

Pages

216-223

Published online

2017-08-28

DOI

10.5603/ARM.2017.0036

Bibliographic record

Adv Respir Med 2017;85(4):216-223.

Keywords

pulmonary hypertension
diffuse parenchymal lung diseases
PAH-specific therapy

Authors

Monika Szturmowicz
Aneta Kacprzak
Jan Kuś

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