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:

1. MMRC dyspnea scale ≥1

2. Threshold FVC 50 - 90% of predicted

3. 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:

1. Prednisone >15 mg/day or changes in prednisone dose in the 8 weeks prior to
screening

2. More than one immune-modulator (i.e., methotrexate, azathioprine, leflunomide,
hydroxychloroquine) or changes in their dosing levels within 12 weeks of
randomization.

3. 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:

1. Absolute neutrophil count (ANC)
2. Thrombocytopenia CTCAE v4.03 Grade 1: Platelets
3. Any active or recurrent bacterial, fungal (with exception of onychomycosis) or
viral infection

4. Presence of human immunodeficiency virus (HIV) infection, hepatitis B or
hepatitis C infections based on screening lab results

5. 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.

6. 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.