Pilot Study to Assess the Efficacy and Safety of H.P. Acthar® Gel in Subjects With Pulmonary Sarcoidosis

This is a Phase 4, multicenter, randomized, double-blind, placebo-controlled pilot study
evaluating the efficacy and safety of Acthar gel in the treatment of pulmonary sarcoidosis.
All subjects who meet eligibility criteria will be randomly assigned to receive either 1 mL
(80 U) of Acthar or 1 mL of a matching placebo subcutaneously (SC) 2 x/week. Subjects will be
assigned to treatment in a 1:1 ratio with up to 50 subjects per arm for a total of up to 100
subjects. Subjects who complete the 24 week randomized, double-blind, placebo-controlled
treatment period will be eligible to continue in an optional 24 week open label extension.

Inclusion Criteria:

- Subject with biopsy confirmed sarcoidosis meeting American Thoracic Society criteria ≥
1 year at screening (Visit 1).

- Symptomatic pulmonary disease defined as any one of the following criteria for
pulmonary function test (PFT); imaging; steroid dose; or symptoms:

1. Decrease ≥ 5% absolute change of % predicted in the best available FVC within the
past 2 years.

2. Decrease ≥ 5% absolute change of % predicted in the best available DLCO within
the past 2 years.

3. Radiographic progression in chest imaging on side by side comparison within the
past 2 years.

4. Progression of pulmonary sarcoidosis necessitating increase in antisarcoidosis
therapy in the past 2 years.

5. Dyspnea with Medical Research Council Scale of ≥ 1 at screening (Visit 1).

- Subject receiving ≥ 5 mg and ≤ 40 mg prednisone (or equivalent) for pulmonary
sarcoidosis.

- Stable prednisone dose ≥ 4 weeks prior to screening (Visit 1).

- Subjects treated with any disease modifying antisarcoidosis drugs (eg, methotrexate)
must be on stable dose for ≥ 3 months prior to screening (Visit 1).

- Forced vital capacity (FVC) ≥ 45% ≤ 95% predicted.

- Diffusing capacity of the lung for carbon monoxide (DLCO) ≥ 30%.

Exclusion Criteria:

- Any difference >10% in FVC on spirometry between the determination at screening (Visit
1) and the determination at Visit 2.

- Pulmonary arterial hypertension requiring or receiving treatment.

- Extra pulmonary involvement that per the Investigator's evaluation requires treatment
that would impede corticosteroid tapering for pulmonary involvement.

- Treatment with antitumor necrosis factor-α antibody (eg, infliximab, adalimumab) in
prior 3 months.