Safety and Tolerability of Pirfenidone in Patients With Systemic Sclerosis−Related Interstitial Lung Disease (SSc-ILD) (LOTUSS)

This study is a Phase 2, multinational, open-label, randomized, parallel-group, safety and
tolerability study of pirfenidone in patients with systemic sclerosis−related interstitial
lung disease (SSc-ILD)

Inclusion Criteria:

1. Diagnosis of SSc confirmed by the American College of Rheumatology classification
criteria of systemic sclerosis (Masi 1980); duration of diagnosis <7 years

2. Diagnosis of SSc-ILD based on an HRCT scan

3. Screening FVC ≥50% of the predicted value, and screening DLCO ≥40% of the predicted
value

4. At study entry, the patient either is not taking SSc-ILD medication or is taking
cyclophosphamide or mycophenolate mofetil

Exclusion Criteria:

1. Clinically significant pulmonary hypertension

2. Known underlying liver disease

3. Clinical evidence of significant aspiration or uncontrolled gastroesophageal reflux

4. History of clinically significant asthma or chronic obstructive pulmonary disease

5. Active infection

6. Diagnosis of another connective tissue disorder

7. Evidence of a malignancy that is likely to result in significant disability or
require significant medical or surgical intervention

8. History of unstable or deteriorating cardiac or pulmonary disease (other than
SSc-ILD)

9. Pregnancy or lactation

10. Creatinine clearance <40 mL/min

11. Prior use of pirfenidone

12. Unsuitable for enrollment or unlikely to comply with study requirements.