Health Condition 1: null- systemic sclerosis with interstitial lung disease
Conditions
Interventions
Intervention1: pirfenidone: one
tablet thrice a day (600 mg/day). The dose will be increased to 2 tablets thrice a day (1200
mg/day) after 1 week of initiating treatment, 3 tablets thrice a day (1800
Sponsors
Post graduate institute of medical education and research
Eligibility
Inclusion criteria
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 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
Exclusion criteria: Change in immunosuppressant drugs (except low dose steroids i.e. prednisolone equivalent 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 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)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| To assess improvement, stabilisation or worsening of interstitial lung disease as measured by change in FVC at 6 months after initiation of therapy with pirfenidone or placebo. Improvement will be defined as improvement in FVC by more than 10%, stabilisation defined as increase or decrease in FVC between 1-10% & worsening will be defined as decline in FVC by more than 10%.Timepoint: 6 months | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. To compare the change in Modified Rodnan skin Score (mRSS) from baseline after 6 months of therapy with pirfenidone or placebo 2. To compare the improvement in 6MWT after 6 months of therapy with pirfenidone or placebo. 3. To compare the change in Mahlerâ??s transient dyspnea index (TDI/BDI) after 6 months of therapy with pirfenidone or placebo. 4. To compare the change in the levels of TGF-β & TNF-α in the serum after 6 months of therapy with pirfenidone or placebo.Timepoint: 6 months | — |
Countries
India
Contacts
Public ContactNupoor Acharya
PGIMER
Outcome results
None listed