title: Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis.
abstract: OBJECTIVE
  Systemic sclerosis (SSc) is characterized by abnormal fibrosis of the skin and internal organs, particularly the lungs. Recent reports have revealed a lack of correlation between bronchoalveolar lavage (BAL) variations and response to cyclophosphamide (CYC) in patients with scleroderma-related alveolitis. Our study aimed to evaluate whether the normalization of BAL cellularity correlates with long-term response to CYC.
  
  
  METHODS
  We retrospectively studied 26 consecutive SSc patients with alveolitis diagnosed by BAL and treated with CYC therapy (cumulative dosage 26.5 +/- 11.7 g; 21.1 +/- 8.9 months of treatment). We evaluated high-resolution computed tomography (HRCT), forced vital capacity (FVC), and carbon monoxide diffusing capacity (DLCO) variations before and after CYC. Radiological and functional parameters were re-evaluated in 23 patients after 1-year follow-up.
  
  
  RESULTS
  BAL cellularity normalized after CYC therapy in 12/26 (46.2%) patients (group 1), while it remained abnormal in 14/26 (53.8%) (group 2). FVC and DLCO of group 1 slightly increased after CYC (p = 0.014 and p = 0.07, respectively) and remained stable at follow-up, whereas in group 2 they did not change after CYC and at follow-up (p = not significant). Moreover, at the end of CYC, FVC and/or DLCO showed a clinical improvement/stabilization in all patients of group 1 versus 8/14 of group 2, while at the re-evaluation 1 year after completing CYC, 2/11 patients of group 1 worsened versus 5/12 of group 2. HRCT progression was observed in 1/11 of group 1 and 8/12 of group 2 (p = 0.009).
  
  
  CONCLUSIONS
  BAL fluid normalization after CYC therapy correlated with long-term response to treatment, contrary to what is observed in individuals with persistent alveolitis.
authors.affiliation: Rheumatology Unit, University of Modena and Reggio Emilia Medical School, Modena Polyclinic, Modena, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Colaci
authors.forename: M
authors.initials: M
authors.lastname: Sebastiani
authors.forename: D
authors.initials: D
authors.lastname: Giuggioli
authors.forename: A
authors.initials: A
authors.lastname: Manfredi
authors.forename: P
authors.initials: P
authors.lastname: Spagnolo
authors.forename: F
authors.initials: F
authors.lastname: Luppi
authors.forename: L
authors.initials: L
authors.lastname: Richeldi
authors.forename: Clodoveo
authors.initials: C
authors.lastname: Ferri
mesh_terms: D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use; D018893:Bronchoalveolar Lavage; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014797:Vital Capacity
chemical_list: D018501:Antirheumatic Agents; D003520:Cyclophosphamide