Study of Efficacy, Safety and Tolerability of ACZ885 (Canakinumab) in Patients With Pulmonary Sarcoidosis

Key Inclusion Criteria:

• Male and female subjects ages 18 to 80 years of age (both inclusive)
• Pulmonary sarcoidosis disease duration of ≥1 year
• Clinically active disease demonstrated either by a biopsy (any organ) or by bronchoalevolar lavage (lymphocytosis >15%, CD4+/CD8+ ration>3.5, CD103+/CD4+/CD4+ ratio <0.2). Patients must also have all of the following criteria:

• MMRC dyspnea scale ≥1
• Threshold FVC 50 - 90% of predicted
• Evidence of parenchymal lung involvement by HRCT at screening or by historical radiological evidence (e.g. CT, MRI or x-ray)

Key Exclusion Criteria:

• Treated pulmonary hypertension
• Previous exposure to concomitant treatment according to the following criteria:

• Prednisone >15 mg/day or changes in prednisone dose in the 8 weeks prior to screening
• More than one immune-modulator (i.e., methotrexate, azathioprine, leflunomide, hydroxychloroquine) or changes in their dosing levels within 12 weeks of randomization.
• Mycophenolate use within 12 weeks of randomization
• Prior treatment with any biologic drug targeting the immune system within 180 days of randomization or history of any previous use of rituximab
• History of bleeding disorder
• Forced vital capacity (FVC) <50% of predicted
• Extra-pulmonary sarcoidosis as primary treatment indication (e.g., involving brain, heart, eye and renal disease with significant hypercalcemia)
• Any conditions or significant medical problems which in the opinion of the investigator immune-compromise the patient and/or places the patient at unacceptable risk for immunomodulatory therapy, such as:

• Absolute neutrophil count (ANC) <LLN (1,500/μl)
• Thrombocytopenia CTCAE v4.03 Grade 1: Platelets <LLN (75.0 x 10exp9/L)
• Any active or recurrent bacterial, fungal (with exception of onychomycosis) or viral infection
• Presence of human immunodeficiency virus (HIV) infection, hepatitis B or hepatitis C infections based on screening lab results
• Presence of active or latent tuberculosis (Tb). If historical Tb result is available, Tb status needs to be confirmed pre-randomization as determined by screening laboratory measurements.
• Clinical evidence or history of multiple sclerosis or other demyelinating diseases, or Felty's syndrome
• Live vaccinations within 3 months prior to the start of the trial
• Current severe progressive or uncontrolled disease which in the judgment of the clinical investigator renders the patient unsuitable for the trial
• Women of child-bearing potential, defined as all women physiologically capable of becoming pregnant, unless they are using methods of contraception defined in the protocol for the study.