STX-100 in Patients With Idiopathic Pulmonary Fibrosis (IPF)

This study was previously posted by Stromedix, Inc. In April, 2014, sponsorship of the trial
was transferred to Biogen. The study drug name was changed from STX-100 to BG00011 and the
study number was changed from STX-003 to 203PF201, to align with sponsor conventions.

Key Inclusion Criteria:

1. Clinical features consistent with IPF prior to screening (based on the American
Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society
(JRS)/Latin American Thoracic Association (ALAT) consensus criteria for the diagnosis
of IPF).

2. Forced (expiratory) Vital Capacity (FVC) ≥ 50% of predicted value.

3. DLco (corrected for hemoglobin) ≥ 30% predicted value.

4. Oxygen saturation > 90% at rest by pulse oximetry while breathing ambient air or
receiving ≤2 L/minute of supplemental oxygen.

5. Residual volume ≤ 120% predicted value.

6. Ratio of Forced Expiratory Volume over 1 second (FEV1) to FVC ≥ 0.65 after the use of
a bronchodilator.

7. Other known causes of interstitial lung disease have been excluded (e.g., drug
toxicities, environmental exposures, connective tissue diseases).

8. High Resolution Computed Tomography (HRCT) image fulfills the criteria for 'Usual
Interstitial Pneumonia (UIP) pattern'.

9. If the HRCT image does not fulfill the criteria for 'UIP pattern' a surgical lung
biopsy is necessary for the diagnosis of IPF (lung biopsy performed prior to screening
is acceptable). If a lung biopsy has been performed, it must fulfill the
histopathological criteria for either 'UIP pattern' or 'probable UIP pattern' with the
appropriate HRCT correlate.

10. Adequate bone marrow and liver function.

11. Patient has a life expectancy of at least 12 months.

Key Exclusion Criteria:

1. Findings that are diagnostic of a condition other than UIP on surgical lung biopsy
(performed either before or after screening), HRCT imaging, transbronchial lung
biopsy, or bronchoalveolar lavage (BAL).

2. Serious local infection or systemic infection within 3 months prior to screening.

3. Treatment with another investigational drug, investigational device, or approved
therapy for investigational use within 4 weeks of initial screening.

4. Currently receiving high dose corticosteroid, cytotoxic therapy (e.g., chlorambucil,
azathioprine, cyclophosphamide, methotrexate), nintedanib (Ofev®), vasodilator therapy
for pulmonary hypertension (e.g., bosentan), unapproved and/or investigational therapy
for IPF or administration of such therapeutics within 5 half-lives of the agent prior
to initial screening in this study.

5. End-stage fibrotic disease requiring organ transplantation within 6 months

NOTE: Other protocol defined Inclusion/Exclusion Criteria may apply.