title: A long-term prospective randomized controlled study of non-specific interstitial pneumonia (NSIP) treatment in scleroderma.
abstract: The association of cyclophosphamide (CYC) and prednisone (PRED) for the treatment of lung fibrosis in systemic sclerosis (SSc) was only evaluated in uncontrolled studies, although in idiopathic interstitial lung disease (ILD) this association seems to be beneficial in patients with non-specific interstitial pneumonia (NSIP).
  
  
  OBJECTIVE
  To treat SSc-ILD in a prospective open-label controlled study based on lung pattern during 12 months of treatment.
  
  
  METHODS
  A 3-year analysis was also performed. Twenty-four consecutive patients with SSc and ILD were submitted to an open lung biopsy. Eighteen patients (NSIP) were randomized in two groups: CYC versus CYC + PRED during 12 months. Lung function tests (diffusion lung capacity of monoxide carbone corrected for hemoglobin concentration (DLCO-Hb), forced vital capacity (FVC), total lung capacity) and Modified Rodnan Skin Score (MRSS) were performed before, after one of treatment and after 3 years from the end of the treatment.
  
  
  RESULTS
  Pulmonary function tests were similar in both groups on baseline. After 1 year of treatment, FVC% was comparable between CYC groups (p = 0.72) and in CYC + PRED (p = 0.40). Three years after the end of treatment, FVC% values (p = 0.39 in group CYC and p = 0.61 in CYC + PRED and p = 0.22 in CYC + PRED) and DLCO-Hb (p = 0.54 in CYC and p = 0.28 in CYC + PRED) were similar compared to 1 year of treatment. We observed a reduction of the MRSS in the CYC + PRED group after 1 year of treatment (p = 0.02); although after 3 years, MRSS values remained stable in both groups.
  
  
  CONCLUSIONS
  CYC was effective to stabilize lung function parameters in NSIP lung pattern of SSc disease for 3 years after the end of a 1-year therapy.
authors.affiliation: Division of Rheumatology, University of São Paulo, São Paulo, Brazil.
authors.forename: Diogo S
authors.initials: DS
authors.lastname: Domiciano
authors.forename: Eloisa
authors.initials: E
authors.lastname: Bonfá
authors.forename: Claudia T L
authors.initials: CT
authors.lastname: Borges
authors.forename: Ronaldo A
authors.initials: RA
authors.lastname: Kairalla
authors.forename: Vera L
authors.initials: VL
authors.lastname: Capelozzi
authors.forename: Edwin
authors.initials: E
authors.lastname: Parra
authors.forename: Romy Beatriz
authors.initials: RB
authors.lastname: Christmann
mesh_terms: D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use*; D011446:Prospective Studies; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone