Health Condition 1: M340- Progressive systemic sclerosis
Conditions
Interventions
Intervention1: Cyclophosphamide: It is an alkylating agent that is cytotoxic to both resting and dividing lymphocytes.
750mg Intravenous pulse monthly for 6 months.
Intervention2: Rituximab: Rituximab
Sponsors
All India Institute of Medical Sciences
Eligibility
Inclusion criteria
Inclusion criteria: 1. Patients with a diagnosis of systemic sclerosis as per the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria, irrespective of their gender, will be included in the study. They may have either diffuse cutaneous or limited cutaneous systemic sclerosis. 2. Age >=18 years. 3. Patients with the following evidence of pulmonary involvement: a)% predicted FVC less than or equal to 70% and/ or b)HRCT chest showing involvement of more than or equal to 20% of lung area with interstitial lung disease 4.Patients already on immunosupressives, who have a baseline and a follow-up PFT and HRCT available
Exclusion criteria
Exclusion criteria: 1. Patients with overlap syndrome. 2. Diagnosis of clinically significant resting pulmonary hypertension requiring treatment diagnosed on echocardiography (defined as tricuspid regurgitation velocity more than 4m/s, equivalent to estimated pulmonary artery systolic pressure of 64 mm Hg) while evaluation of the patient during study. 3. Evidence of uncontrolled congestive heart failure, unstable ischemic heart disease, history of pulmonary embolism, or cardiac arrhythmia requiring chronic anticoagulation. 4. FVC less than or equal to 15% at baseline. 5. More than 50% area of lung showing fibrosis on HRCT chest at baseline. 6. Hematologic abnormality at screening including: a) Leukopenia (white blood cells [WBC] b) Thrombocytopenia (platelet count c) clinically significant anemia (hemoglobin d) Participants with an identified and correctable etiology will be eligible if repeat testing shows values greater than the above mentioned cut-off. 7. A diagnosis of chronic liver disease or abnormal baseline liver function tests (total bilirubin or liver enzymes, alanine aminotransferase and aspartate aminotransferase > 2.0 times the upper normal limit). 8. Serum creatinine >2.0mg/dl 9. Pregnancy and/or breast feeding 10. If of child bearing potential (a female participant less than 55 years of age who has not been postmenopausal for more than 5 years or who has not had a hysterectomy and/or oophorectomy), failure to employ reliable means of contraception. 11. Prior use of oral or intravenous cyclophosphamide, MMF, azathioprine or other putative disease modifying medications in last 3 months (assessed at baseline). 12. Current use, or use within the 30 days prior to their baseline visit, of prednisone (or equivalent) in dose of 40 mg/day 13. Active infection (Hepatitis B, Hepatitis C, tuberculosis, HIV) whose management would be compromised by immunosuppression.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1.To measure absolute and percentage rate of change in PFT (DLCO and FVC) from baseline to 1 year of follow-up 2.To assess the changes from baseline in HRCT chest patterns and quantitative HRCT scores for interstitial lung disease at 1 year of follow-up 3. To study the extent of lung involvement functionally (PFT) and radiologically (HRCT). Timepoint: 1 year | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. To measure absolute and percentage change in MRSS score from baseline to 1 year of follow-up 2. To study effect of immunosuppressive therapy on frequency of a)Raynaudâ??s Phenomenon b)Digital ulceration 3. To assess correlation between PFT(FVC,DLCO) and HRCT scores 4. To assess change in 6-minute walk test at end of 1 year as compared to baseline. Timepoint: 1 year | — |
Countries
India
Contacts
Public ContactDr Mohammed Thalha
All India Institute of Medical Sciences
Outcome results
None listed