title: High-dose prednisolone and bolus cyclophosphamide in interstitial lung disease associated with systemic sclerosis: a prospective open study.
abstract: OBJECTIVE
  Currently, therapy for interstitial lung disease in patients with systemic sclerosis is unsatisfactory. A prospective open label study was conducted in a North Indian tertiary Institute to assess the efficacy of intermittent pulse cyclophosphamide (CYC) and high-dose prednisolone in systemic sclerosis (SSc)-related interstitial lung disease (ILD).
  
  
  METHODS
  Consecutive patients with SSc and ILD, diagnosed on spirometry, carbon monoxide diffusing capacity (DLCO) and high-resolution computed tomography (HRCT) scan were treated. Pulmonary function tests were carried out at baseline and after 6 months. Patients received oral prednisolone 1 mg/kg body weight initially, with tapering to a dose of 7.5 mg/day was reached. Monthly CYC pulses were given for 6 months followed by 3-monthly maintenance pulses. CYC was discontinued in patients with declining pulmonary function, adverse effects or static disease after 6 months.
  
  
  RESULTS
  Average disease duration of 36 patients was 59.78 +/- 63.22 months. Seven patients improved (forced vital capacity [FVC] increase 10% or DLCO increase 15%), five deteriorated (FVC decline 10% or DLCO decline 15%) and 24 had stable disease. Thus, 31 out of 36 patients either improved or had static lung disease. Mean FVC (% of predicted) improved by 4.16% over 6 months (P = 0.069). Mean DLCO (% of predicted) improved by 5.66% (P = 0.27). Average % of predicted DLCO at baseline was 39%.
  
  
  CONCLUSIONS
  High-dose prednisolone with pulse CYC can either improve or stabilize lung functions in patients with severe systemic sclerosis lung disease irrespective of presence of ground glass appearance on HRCT.
authors.affiliation: Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India. awanchu@yahoo.com
authors.forename: Ajay
authors.initials: A
authors.lastname: Wanchu
authors.forename: Bettadpura Shamanna
authors.initials: BS
authors.lastname: Suryanaryana
authors.forename: Shefali
authors.initials: S
authors.lastname: Sharma
authors.forename: Aman
authors.initials: A
authors.lastname: Sharma
authors.forename: Pradeep
authors.initials: P
authors.lastname: Bambery
mesh_terms: D000328:Adult; D002248:Carbon Monoxide / Q000378:metabolism; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage*; D011446:Prospective Studies; D020551:Pulse Therapy, Drug; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy*; D016896:Treatment Outcome; D014797:Vital Capacity
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D002248:Carbon Monoxide; D003520:Cyclophosphamide; D011239:Prednisolone