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Study breakdown

Inhaled Treprostinil Successfully Treated Pulmonary Hypertension in Severe Lung Disease

Case ReportPreliminary evidence
The takeaway

A patient with severe interstitial pneumonia and pulmonary hypertension showed significant improvement with inhaled treprostinil, a prostacyclin analog.

64 mmHg

Initial tricuspid regurgitation pressure gradient before treatment

What the researchers found

Inhaled treprostinil improved BNP levels, tricuspid pressure gradient, and oxygen requirements in a patient with severe interstitial pneumonia and pulmonary hypertension.

Why it matters

Demonstrates real-world effectiveness of inhaled treprostinil in a difficult-to-treat patient with combined severe lung disease and pulmonary hypertension.

The numbers in context

- Patient age: 72 years

- Maximum TRPG before treatment: 64 mmHg

- BNP levels and TRPG improved after inhaled treprostinil

- Oxygen requirement decreased after treatment

How the study worked

Single case report of a 72-year-old male patient.

Who was studied

Single 72-year-old male patient with interstitial pneumonia and Group 3 pulmonary hypertension, Japan.

What this study cannot tell us

Single case report cannot establish efficacy. Spontaneous improvement or concurrent treatments may have contributed.

How to read the evidence

Single case report. Lowest level of clinical evidence but useful for illustrating treatment response.

When this study was published

Published in 2025.

The bigger picture

Inhaled treprostinil is an approved therapy for group 3 pulmonary hypertension, and case reports like this add to the growing evidence of its clinical utility in severe lung disease.

Questions still open

  • How durable is the response to inhaled treprostinil in interstitial lung disease?
  • Should all patients with severe ILD-related PH receive inhaled treprostinil?

Common questions

What is inhaled treprostinil?
A prostacyclin analog delivered by inhalation that dilates pulmonary blood vessels, reducing pressure in the lungs and improving heart function.
Is this treatment standard for lung disease patients with pulmonary hypertension?
Inhaled treprostinil is the recommended first-choice drug for group 3 pulmonary hypertension (PH associated with chronic lung disease).

Read the original research

Pulmonary Hypertension Associated with Severe Interstitial Pneumonia Successfully Treated with Inhaled Treprostinil: A Case Report.

Internal medicine (Tokyo, Japan)

Citation

Otsuka, Mitsuki; Sonoda, Shiro; Yamada, Takayuki; Aoki, Shotaro; Yasuda, Tomoka; Sawada, Atsushi; Shirai, Tsuyoshi; Tateishi, Tomoya; Furusawa, Haruhiko; Miyazaki, Yasunari. (2025). Pulmonary Hypertension Associated with Severe Interstitial Pneumonia Successfully Treated with Inhaled Treprostinil: A Case Report.. Internal medicine (Tokyo, Japan). https://doi.org/10.2169/internalmedicine.6206-25