Safety and Tolerability Study of Macitentan in Patients With Idiopathic Pulmonary Fibrosis (MUSIC OL)

Long Term, Single-arm, Open-label Extension Study of the MUSIC Study to Assess the Safety and Tolerability of Macitentan in Patients With Idiopathic Pulmonary Fibrosis

The MUSIC OL study is an open-label extension study, in which all eligible patients having completed the double-blind AC-055B201/MUSIC study as scheduled receive macitentan 10 mg once daily. The study objective is to assess the long-term safety and tolerability of macitentan in patients with idiopathic pulmonary fibrosis (IPF).

Study Overview

• Status: Withdrawn
• Conditions: Idiopathic Pulmonary Fibrosis
• Intervention / Treatment: Drug: Macitentan

• Study Type: Interventional
• Phase: Phase 2

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

• Signed informed consent prior to initiation of any study-related procedure.
• Patients with IPF and having completed the double-blind AC-055B201/MUSIC study as scheduled, i.e., having remained in the study until the sponsor-declared end-of-study (EOS), whether or not study treatment was prematurely discontinued.
• Women of childbearing potential must have a negative pre-treatment serum pregnancy test and must use a reliable method of contraception during study treatment and for at least 28 days after study treatment termination.

Exclusion Criteria:

• Any major violation of protocol AC-055B201/MUSIC.
• Premature discontinuation of study treatment during the AC-055B201/MUSIC study due to an adverse event (AE) assessed as related to the use of macitentan, excluding events of IPF worsening.
• Pregnancy or breast-feeding.
• AST and/or ALT > 3 times the upper limit of the normal range.
• Any known factor or disease that might interfere with treatment compliance, study conduct or interpretation of the results, such as drug or alcohol dependence or psychiatric disease.
• Known hypersensitivity to drugs of the same class as macitentan, or any of the excipients.

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Macitentan; Macitentan tablet, 10 mg, once daily	Drug: Macitentan; 10 mg, tablet, once daily

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Adverse Events leading to premature discontinuation of study drug	5 years

Secondary Outcome Measures

Outcome Measure	Time Frame
Treatment-emergent Severe Adverse Events & occurrence of AST and/or ALT > 3 times the upper limit of the normal range up to 28 days after study drug discontinuation	5 years

Collaborators and Investigators

• Sponsor: Actelion
• Investigators: Study Chair: Loic Perchenet, PhD, Actelion

Study record dates

Study Major Dates

• Study Start (Actual): July 31, 2011
• Primary Completion (Estimated): August 31, 2016
• Study Completion (Estimated): August 31, 2016

Study Registration Dates

• First Submitted: May 2, 2011
• First Submitted That Met QC Criteria: May 3, 2011
• First Posted (Estimated): May 4, 2011

Study Record Updates

• Last Update Posted (Actual): June 22, 2025
• Last Update Submitted That Met QC Criteria: June 20, 2025
• Last Verified: June 1, 2025

More Information

Terms related to this study

• Keywords: Idiopathic pulmonary fibrosis; IPF; pulmonary fibrosis
• Additional Relevant MeSH Terms: Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Lung Diseases, Interstitial; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis; Fibrosis; Molecular Mechanisms of Pharmacological Action; Endothelin A Receptor Antagonists; Endothelin Receptor Antagonists; Endothelin B Receptor Antagonists; Macitentan
• Other Study ID Numbers: AC-055B202