title: Effect of Sequential Intravenous Pulse Cyclophosphamide-Azathioprine in Systemic Sclerosis-Interstitial Lung Disease: An Open-Label Study.
abstract: Systemic sclerosis (SSc) is a rare connective tissue disorder of unknown aetiology. Pulmonary involvement
  contributes substantially to its morbidity and mortality. Treatment of pulmonary disease due to SSc remains unsatisfactory.
  We examined the effect of sequential six-month intravenous pulse therapy with cyclophosphamide (CYC) followed by
  azathioprine and low-dose corticosteroids on SSc associated interstitial lung disease (SSc-ILD).
  
  
  
  In a single-centre, prospective, observational, open-labelled study; nine patients (eight females, one male) with
  SSc-ILD were treated with intravenous pulse CYC (600mg/m(2) body surface area) at monthly interval for six cycles with
  oral prednisolone 10mg daily. Subsequently, azathioprine (2-3mg/Kg) was administered while continuing with the same
  dose of prednisolone. Primary end-points were forced vital capacity (FVC) and high resolution computed tomography
  (HRCT) scan of thorax score. Secondary end-points were quality of life measured by health assessment questionnaire-disability
  index (HAQ-DI) and six-minute walk distance (6WMD) test.
  
  
  
  After one year of observation, the FVC showed significant improvement (p=0.003). The 6WMD also improved
  significantly (p=0.0028). However, change in HRCT scan scoring and HAQ-DI score was not significant.
  
  
  
  Intravenous, pulse CYC followed by azathioprine along with low-dose corticosteroids produces significant
  improvement in FVC and 6WMD at 12-month follow-up without significant change in radiological manifestations and health status.
authors.forename: Susmita
authors.initials: S
authors.lastname: Kundu
authors.forename: Suman
authors.initials: S
authors.lastname: Paul
authors.forename: K
authors.initials: K
authors.lastname: Hariprasath
authors.forename: Rajesh
authors.initials: R
authors.lastname: Agarwal
authors.forename: Saswata
authors.initials: S
authors.lastname: Ghosh
authors.forename: Debabani
authors.initials: D
authors.lastname: Biswas
mesh_terms: D000328:Adult; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D011446:Prospective Studies; D013902:Radiography, Thoracic; D012595:Scleroderma, Systemic / Q000150:complications*; D014797:Vital Capacity; D000070857:Walk Test
chemical_list: D003520:Cyclophosphamide; D011239:Prednisolone; D001379:Azathioprine