title: Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis.
abstract: BACKGROUND
  Lung inflammation (alveolitis) may cause lung fibrosis in scleroderma.
  
  
  OBJECTIVE
  To determine whether cyclophosphamide treatment is associated with retention of lung function and improved survival in scleroderma patients with alveolitis.
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  Johns Hopkins and University of Maryland Scleroderma Center.
  
  
  METHODS
  103 patients with scleroderma who had bronchoalveolar lavage or lung biopsy.
  
  
  METHODS
  Cyclophosphamide therapy.
  
  
  METHODS
  1) Serial measurement of forced vital capacity (FVC) and carbon monoxide diffusing capacity and 2) survival.
  
  
  RESULTS
  During a median follow-up of 13 months after bronchoalveolar lavage or biopsy, patients with alveolitis who did not receive cyclophosphamide therapy experienced a decrease in FVC (mean difference, -0.28 L [95% Cl, -0.41 to -0.16 L] and -7.1% of the predicted value [Cl, -10.9% to -4.0%]). Carbon monoxide diffusing capacity also decreased in these patients (mean difference, -3.3 x mmol min(-1) x kPa(-1) [Cl, -4.6 to -2.1 mmol x min(-1) x kPa(-1)] and -9.6% of the predicted value [Cl, -16.7% to -2.4%]). During a median follow-up of 16 months, patients with alveolitis who received cyclophosphamide were more likely to have a good outcome (stabilization or improvement) in FVC (relative risk, 2.5 [Cl, 1.5 to 4.1]) and diffusing capacity (relative risk, 1.5 [Cl, 1.0 to 2.2]). These patients also had improved survival; the median survival rate was 89% (25th, 75th percentiles, 84%, 94%) compared with 71% (25th, 75th percentiles, 55%, 86%) in untreated patients (P = 0.01, log-rank test).
  
  
  CONCLUSIONS
  The presence of lung inflammation identifies patients with scleroderma who are more likely to have worsening lung function. Lung function outcomes and survival are improved in patients with alveolitis who receive cyclophosphamide.
authors.affiliation: Veterans Affairs Maryland Health Care System, University of Maryland School of Medicine, and Johns Hopkins University Medical Institutions, Baltimore, USA.
authors.forename: B
authors.initials: B
authors.lastname: White
authors.forename: W C
authors.initials: WC
authors.lastname: Moore
authors.forename: F M
authors.initials: FM
authors.lastname: Wigley
authors.forename: H Q
authors.initials: HQ
authors.lastname: Xiao
authors.forename: R A
authors.initials: RA
authors.lastname: Wise
mesh_terms: D001706:Biopsy; D018893:Bronchoalveolar Lavage; D002248:Carbon Monoxide; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011014:Pneumonia / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology; D011650:Pulmonary Alveoli / Q000503:physiopathology; D011653:Pulmonary Diffusing Capacity; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality; D016896:Treatment Outcome; D014797:Vital Capacity
chemical_list: D007166:Immunosuppressive Agents; D002248:Carbon Monoxide; D003520:Cyclophosphamide