A randomized, double-blind, placebo-controlled, multipledose, exploratory proof of concept study to assess the safety, tolerability, efficacy, pharmacodynamics and

INTERVENTION: Product Code: QAX576 Pharmaceutical Form: Powder and solvent for solution for infusion Current Sponsor code: QAX576 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150‐ Pharmaceutical form of the placebo: Powder and solvent for solution for infusion Route of administration of the placebo: Intravenous use CONDITION: Patients with rapidly progressive idiopathic pulmonary fibrosis ; MedDRA version: 14.1 Level: PT Classification code 10021240 Term: Idiopathic pulmonary fibrosis System Organ Class: 10038738 ‐ Respiratory, thoracic and mediastinal disorders PRIMARY OUTCOME: Main Objective: • To evaluate the safety and tolerability of multiple intravenous dosing of QAX576 in; patients with IPF; • To evaluate the effect of multiple intravenous dosing of QAX576 on lung function; assessed by FVC at 1 year as compared to baseline Primary end point(s): Primary endpoint:; • The primary efficacy endpoint is change from baseline in FVC measurement at 52 weeks as assessed at each clinical visit post‐baseline.; Additional details are included in section 9.4 of the clinical study protocol; ; Secondry endpoints:; • all‐cause mortality, time to clinical worsening defined as 10% fall in FVC or 15% fall in DLco, lung transplant or lung disease (IPF)‐related death.; • incidence of exacerbation of IPF.; • lung volume (TLC, RV, FRV) and diffusing capacity of the lung for carbon monoxide (DLco).; • 6 minute walk distance, pulse oximetry and heart rate recovery.; • progression of fibrosis in the lungs as measured by Quantitative High Resolution; Computerized Tomography (HRCT).; • patient reported symptoms. Secondary Objective: 1. To evaluate the effect of multiple intravenous dosing of QAX576 on additional measures of clinical efficacy, including:; • all‐cause mortality, time to clinical worsening defined as 10% fall in FVC or 15% fall; in DLco, lung transplant or lung disease (IPF)‐related death.; • incidence of exacerbation of IPF.; • lung volume (TLC, RV, FRV) and diffusing capacity of the lung for carbon monoxide; (DLco).; • 6 minute walk distance, pulse oximetry and heart rate recovery.; • progression of fibrosis in the lungs as measured by Quantitative High Resolution; Computerized Tomography (HRCT).; • patient reported symptoms.; 2. To evaluate the pharmacokinetics of QAX576 in this patient population. INCLUSION CRITERIA: 1. Written informed consent must be obtained before any participation in the study. 2. Male or female subjects of =18 years of age to =80 years of age. 3. Diagnosis of IPF, based on an appropriate clinical definition of IPF as detailed in the (ATS/ERS/JRS/ALAT statement: Idiopathic Pulmonary Fibrosis:Evidence‐based Guidelines for diagnosis and management (Raghu G et al 2011) Diagnosis must be confirmed by diagnostic HRCT or lung biopsy 4. A 6‐minute walk test (6MWT) distance =50 meters at Screening (use of supplemental oxygen allowed). 5. Forced Vital Capacity FVC) =50% of predicted at Screening. 6. Diffusing capacity for carbon monoxide (DLco) uncorrected =30 of predicted at Screening 7. Body mass index (BMI) must be within the range 18 to 40 8. Subjects should be able to communicate well with the investigator and to understand and comply with the requirements of the study. 9. Female patients must be of non‐childbeari