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


NCT ID: NCT01933334
Sponsor Protocol Number: PSSc-001

About this study

PSSc-001 (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).

Participation eligibility

Participant eligibility includes age, gender, type and stage of disease, and previous treatments or health concerns. Guidelines differ from study to study, and identify who can or cannot participate. There is no guarantee that every individual who qualifies and wants to participate in a trial will be enrolled. Contact the study team to discuss study eligibility and potential participation.

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.

More information


  • A multicenter, ongoing study of early-diagnosed cases of systemic sclerosis and comparison patients with systemic lupus erythematosus, polymyositis/dermatomyositis, and Raynaud's phenomenon was conducted in order to develop classification criteria for systemic sclerosis. Preliminary criteria are proposed namely, the finding of either the sole major criterion, i.e., proximal scleroderma, or two or more of the minor criteria, i.e., 1) sclerodactyly, 2) digital pitting scars of fingertips or loss of substance of the distal finger pad, and 3) bilateral basilar pulmonary fibrosis. When applied to the case and comparison patients included in this study, these proposed criteria had a 97% sensitivity for definite systemic sclerosis and 98% specificity. Read More on PubMed

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