Safety and Tolerability Study of Macitentan in Patients With Idiopathic Pulmonary Fibrosis

Study Overview
=================
Brief Summary
-----------------
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).

Official Title
-----------------
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

Conditions
-----------------
Idiopathic Pulmonary Fibrosis

Intervention / Treatment
-----------------
* Drug: Macitentan


Participation Criteria
=================
Eligibility Criteria
-----------------
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.

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Macitentan<br>Macitentan tablet, 10 mg, once daily | Drug: Macitentan<br>* 10 mg, tablet, once daily<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

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

Secondary Outcome Measures

| Outcome Measure | Measure Description | 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 | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Idiopathic pulmonary fibrosis, IPF, pulmonary fibrosis