Efficacy and Safety of Riociguat in Patients With Symptomatic Pulmonary Hypertension (PH) Associated With Idiopathic Interstitial Pneumonias (IIP)

Inclusion Criteria

• •Men or women aged from ≥18 to ≤80 years
• •Diagnosed with one of the following (confirmed using a multidisciplinary approach, as per ATS(American Thoracic Society) / ERS(European Respiratory Society) / JRS (Japanese Respiratory Society) / ALAT(Latin American Thoracic Association) guidelines:
• •* Major IIPs (idiopathic interstitial pneumonias) diagnosis or suspected as one of the following:
• •* Idiopathic pulmonary fibrosis
• •* Idiopathic nonspecific interstitial pneumonia
• •* Respiratory bronchiolitis-interstitial lung disease
• •* Desquamative interstitial pneumonia
• •* Cryptogenic organizing pneumonia
• •* Acute interstitial pneumonia
• •* Rare IIPs diagnosis by one of the following:
• •* Idiopathic lymphoid interstitial pneumonia
• •* Idiopathic pleuroparenchymal fibroelastosis
• •* Unclassifiable idiopathic interstitial pneumonias
• •Forced Vital Capacity (FVC) ≥ 45 %
• •6MWD (6 minutes walking distance) ≥ 150 m to ≤ 450 m {under stable O2(oxygen) supplementation via nasal cannula}
• •Diagnosis of PH (pulmonary hypertension) confirmed by right heart catheter (RHC) with (mean artery pulmonary artery pressure )mPAP ≥ 25 mmHg and (pulmonary artery wedge pressure)PAWP ≤15 mmHg at rest
• •Systolic blood pressure (SBP) ≥ 95 mmHg and no signs or symptoms of hypotension
• •WHO functional class II-IV
• •Women of childbearing potential can only be included in the study if a pregnancy test is negative. Women of childbearing potential must agree to use adequate contraception when sexually active. 'Adequate contraception' is defined as any combination of at least 2 effective methods of birth control, of which at least one is a physical barrier (e.g. condoms with hormonal contraception or implants or combined oral contraceptives, certain intrauterine devices). Adequate contraception is required from the signing of the informed consent form up until 4 weeks after the last study drug administration