Study to evaluate if macitentan is efficient and safe enough to be used for treatment of idiopathic pulmonary fibrosis.

A double-blind, randomized, placebo-controlled, multicenter, parallel group study to evaluate the efficacy, safety, and tolerability of macitentan in patients with idiopathic pulmonary fibrosis.

Status

Active, not recruiting

Phases

Phase 2

Study type

Interventional

Source

ANZCTR

Registry ID

ACTRN12609000458235

Acronym

MUSIC: Macitentan USe in an Idiopathic pulmonary fibrosis Clinical study

Enrollment

156

Registered

2009-06-15

Start date

2009-05-27

Completion date

Unknown

Last updated

2026-02-04

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

None listed

Brief summary

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal lung disease with an unknown cause. Outside of Japan, no drug has been approved so far for the treatment of IPF. The study medication that is tested in this research, macitentan, works by blocking the effect of a substance called endothelin, which has been detected in increased amounts in patients with IPF. By blocking the action of endothelin, macitentan may increase the breathing capacity, improve the quality of life of patients and reduce the progression of the disease. The main purpose of this study is to find out if macitentan is efficient and safe enough to be used for treatment of IPF.

Interventions

Macitentan 10 mg, tablet. Taken orally, once daily, every morning throughout Period 1 (12 months) and Period 2 (variable, at least a further 12 months), until the last patient has completed 24 months therapy (Period 1 and 2 combined) unless the study medication is prematurely discontinued.

Study design

Allocation

Randomised controlled trial

Intervention model

Parallel

Primary purpose

Treatment

Masking

Blinded (masking used)

Eligibility

Sex/Gender

All

Age

18 Years to No maximum

Healthy volunteers

Inclusion criteria

- Signed informed consent. - IPF diagnosis within 3 years prior to randomization, proven according to American Thoracic Society/European Respiratory Society (ATS/ERS) consensus conference criteria, with surgical lung biopsy.

Exclusion criteria

- Interstitial lung disease due to conditions other than IPF. - Presence of extensive honeycombing on baseline high resolution computed tomography (HRCT) scan performed within 3 months prior to randomization. - Forced Vital Capacity (FVC) < 50% predicted, or FVC < 1.2 liter. - Diffusing capacity of the lung for carbon monoxide (DLco) < 30% predicted. - Residual volume at least 120% predicted. - Forced expiratory volume of the lung in 1 second (FEV1)/FVC <0.70. - Aspartate aminotransferase (AST) and/or Alanine aminotransferase (ALT) > 1.5 x Upper limit of Normal (ULN). - Hemoglobin < 75% of the lower limit of the normal range. - Systolic Blood Pressure (sBP) < 100 mmHg.

Outcome results