Pirfenidone for Progressive Fibrotic Sarcoidosis

Patients who meet the inclusion and exclusion criteria will be randomized to be treated with
either placebo or pirfenidone at the current approved dosage for idiopathic pulmonary
fibrosis at a 2:1 pirfenidone to placebo ratio. Patients treated with pirfenidone will be
titrated using the following schedule:

One 267 mg capsules three times a day for two weeks. Two 267 mg capsules three times a day
for two weeks Three 267 mg capsules three times a day thereafter Patients will be instructed
to take all doses of medication with food. Dosage will be titrated on an individual basis
depending on patient tolerance of medication.

Those randomized to placebo will receive the same schedule using placebo tablets.

Block randomization will be done at each site. Liver function tests will be evaluated as
listed above. If the patient has an abnormal liver function test, then the dose will be
adjusted per the company's protocol for commercial drug.

Inclusion Criteria:

- Diagnosis of sarcoidosis

- Pulmonary function testing with a Composite Physiologic Index (CPI) score of greater
than 40

- Patient must have evidence of >20% fibrosis on high resolution cat scan

- Patients must be on a stable prednisone therapy for sarcoidosis for at least two
months and no change in other immunosuppressives in the two months prior to entry into
study

- Age greater than 18 and less than 90.

- Able to provide written informed consent for participation in the study

Exclusion Criteria:

- Patients receiving therapy for precapillary pulmonary hypertension.

- Patients with liver disease Childs class 3 or 4

- Patients with a left ventricular ejection fraction of less than 40%

- Patients receiving more than 20 mg prednisone daily or its equivalent

- Patients with massive hemoptysis within prior three months. Patients with mycetomas
are eligible as long as no massive hemoptysis in prior three months.

- Patients with clinically important co-existing disease which in the opinion of the
investigator is likely to affect patient's chance for survival during the course of
the study

- Patient who is pregnant, lactating, intending to become pregnant during the study, or
child bearing capacity who is not willing to use appropriate birth control methods
approved by investigator