title: Usefulness of Cyclophosphamide Pulse Therapy in Interstitial Lung Diseases.
abstract: BACKGROUND
  Interstitial lung diseases (ILDs) are a group of disorders characterised by progressive lung function decline. Stabilisation of lung function under intermittent i.v. cyclophosphamide was shown in patients suffering from systemic sclerosis, yet data in ILD patients are scarce.
  
  
  OBJECTIVE
  To retrospectively evaluate the usefulness of cyclophosphamide pulse therapy in ILD.
  
  
  METHODS
  We retrospectively analysed all patients who received i.v. cyclophosphamide in our centre from 2002 to 2012. Lung function, survival status, and bronchoalveolar lavage cytology were recorded during a follow-up period of 18 months.
  
  
  RESULTS
  Twenty-six patients with idiopathic pulmonary fibrosis, 6 with lymphocytic interstitial pneumonia (LIP), 8 with idiopathic non-specific interstitial pneumonia (NSIP), 7 with rheumatoid arthritis-associated ILD, and 7 with perinuclear anti-neutrophil cytoplasmic antibody-positive ILD (pANCA+ ILD) were included. Patients with LIP and NSIP had the best survival outcome, those with pANCA+ ILD the worst. In the total cohort, we found a significantly higher total lung capacity decline in the year before treatment compared to the year after treatment.
  
  
  CONCLUSIONS
  This retrospective analysis of cyclophosphamide treatment shows a stabilisation of lung function in most patients with fibrotic ILDs, yet prospective studies in clearly defined diagnoses are urgently needed.
authors.affiliation: Department of Pneumology, University Medical Centre, Albert Ludwig University, Freiburg, Germany.
authors.forename: Jonas Christian
authors.initials: JC
authors.lastname: Schupp
authors.forename: Thomas
authors.initials: T
authors.lastname: Köhler
authors.forename: Joachim
authors.initials: J
authors.lastname: Müller-Quernheim
mesh_terms: D000368:Aged; D003520:Cyclophosphamide / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D007262:Infusions, Intravenous; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014109:Total Lung Capacity
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide