to study the efficacy of pirfenidone in systemic sclerosis related interstitial lung disease

Not Applicable

• Conditions: Health Condition 1: null- systemic sclerosis with interstitial lung disease

• Registration Number: CTRI/2018/01/011449
• Lead Sponsor: Post graduate institute of medical education and research

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Last Update Posted: 24 November 2021

Recruitment & Eligibility

• Status: ot Yet Recruiting
• Sex: Not specified
• Target Recruitment: 0

Inclusion Criteria

1.Patients with SSc as diagnosed by the American College of

Rheumatology (ACR) criteria, 2013 & patients with other connective tissue diseases

who, in parallel, meet the ACR criteria for SSc

2. Presence of ILD on HRCT chest

3. FVC >= 50%

4. Duration of SSc for <=7 years, with onset defined as the appearance of the first nonRaynaudâ??s

phenomenon.

5. DLCO 30% to 89% of predicted normal

6. Consenting for participating in study

7. Received no new immunosuppression for last 6 months (on stable doses of

immunosuppressants like azathioprine, MMF, cyclophosphamide, methotrexate for > 6

months)

Exclusion Criteria

Change in immunosuppressant drugs (except low dose steroids i.e. prednisolone

equivalent <=10 mg/day) for ILD in the previous 6 months.

2. Received biologics in the past

3. Persistent leukopenia or thrombocytopenia

4. Pregnant or breastfeeding females

5. Severe PAH (mean pulmonary arterial pressure >55mmHg) requiring drug therapy

6. FEV1/FVC ratio <=65%

7. Uncontrolled congestive heart failure

8. Any other abnormalities noted on chest X-ray or HRCT other than ILD

9. Active infection

10. Inflammatory myopathy

11. Mixed connective tissue disease

12. Smoking during last 6 months

13. Other serious co-morbidities which could compromise the patientâ??s ability to complete

the study

14. Abnormal liver function tests (AST/ALT > 3times, bilirubin >1.5)

Study & Design

• Study Type: Interventional
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
To assess improvement, stabilisation or worsening of interstitial lung disease as <br/ ><br>measured by change in FVC at 6 months after initiation of therapy with pirfenidone or <br/ ><br>placebo. Improvement will be defined as improvement in FVC by more than 10%, <br/ ><br>stabilisation defined as increase or decrease in FVC between 1-10% & worsening will <br/ ><br>be defined as decline in FVC by more than 10%.Timepoint: 6 months

Secondary Outcome Measures

Name	Time	Method
1. To compare the change in Modified Rodnan skin Score (mRSS) from baseline after 6 <br/ ><br>months of therapy with pirfenidone or placebo <br/ ><br>2. To compare the improvement in 6MWT after 6 months of therapy with pirfenidone or <br/ ><br>placebo. <br/ ><br>3. To compare the change in Mahlerâ??s transient dyspnea index (TDI/BDI) after 6 months <br/ ><br>of therapy with pirfenidone or placebo. <br/ ><br>4. To compare the change in the levels of TGF-β & TNF-α in the serum after 6 months of <br/ ><br>therapy with pirfenidone or placebo.Timepoint: 6 months