Scleroderma Lung Study III - Combining Pirfenidone With Mycophenolate

Inclusion Criteria

• Age ≥18 yrs
• Scleroderma as determined by the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria.
• Grade ≥2 on the Magnitude of Task component of the Mahler Modified Dyspnea Index
• FVC-% of ≤85% at screening
• Onset of the first non-Raynaud manifestation of SSc within the prior 84 months.
• Presence of any ground-glass opacification (GGO) on thoracic high-resolution computed tomography (HRCT)
• Repeat FVC-% at the baseline visit within 10% of the FVC-% value measured at screening. If these criteria are not met, a repeat FVC-% may be obtained within 7 days and the subject may qualify for randomization if the repeat FVC-% agrees within 10% of the FVC-% obtained at screening.

Exclusion Criteria

• Disease features supporting the primary diagnosis of another connective tissue disease such as rheumatoid arthritis, systemic lupus erythematosus or mixed connective tissue disease (Features consistent with a secondary Sjogren syndrome or scleroderma-associated myopathy will be allowed).
• FVC-% of 2.0 x upper normal limit
• Serum creatinine >2.0mg/dl
• History of recurrent aspiration, uncontrolled heartburn, or gastroesophageal reflux disease (GERD) with a reflux scale score of >1.00 as determined by a UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Scale (UCLA SCTC GIT), Version 2.0.

Participants with uncontrolled heartburn or GERD that is amenable to medical management may be eligible if repeat testing within the maximal 90-day screening period meets this criteria.

• Known achalasia, esophageal stricture or esophageal dysfunction sufficient to limit the ability to swallow medication.
• Pregnancy (as documented by blood test) and/or breast feeding
• If of child bearing potential (a female participant 10 mg/day.
• Smoking of cigars, pipes, or cigarettes during the past 6 months.
• Use of contraindicated medications, including medications with putative disease-modifying properties that do not meet the exposure limits described in Exclusion Criteria #15 and #16, moderate or strong inhibitors of cytochrome P450 (CYP) isozyme 1A2 (CYP1A2) (note ciprofloxacin allowed up to a dose of 500 mg twice daily), and moderate inducers of CYP1A2 (such as tobacco smoke or phenytoin). See Protocol Section 7.5 for complete list.