title: Chitotriosidase activity in patients with interstitial lung diseases.
abstract: BACKGROUND
  In previous papers, we found significantly higher activity of chitotriosidase, a macrophage derived enzyme, in serum and BAL of patients with sarcoidosis, especially in those with progressing disease and lung involvement, than in controls. Locally and systemically produced chitotriosidase activity was correlated with radiological stage and also with degree of lung infiltration, suggesting that this enzyme may play a role in the pathogenesis of sarcoidosis and may be used as a marker of disease severity.
  
  
  OBJECTIVE
  To analyse chitotriosidase activity in serum and bronchoalveolar lavage of patients with idiopathic pulmonary fibrosis and pulmonary fibrosis associated with systemic sclerosis and to compare it with chitotriosidase activity in controls and sarcoidosis patients.
  
  
  METHODS
  Chitotriosidase activity was determined by a fluorometric assay.
  
  
  RESULTS
  The results showed that serum chitotriosidase activity was only elevated in sarcoidosis patients; in patients with idiopathic pulmonary fibrosis and pulmonary fibrosis associated with systemic sclerosis it was in the normal range. On the contrary, in BAL of sarcoidosis and idiopathic pulmonary fibrosis patients the activity was significantly higher than in controls.
  
  
  CONCLUSIONS
  Serum chitotriosidase is a potential marker of sarcoidosis severity; it increases in sarcoidosis in relation to radiological stage and degree of lung infiltration. The increase in chitotriosidase activity in BAL of sarcoidosis and idiopathic pulmonary fibrosis patients suggests that the enzyme could be involved in fibrogenesis in diffuse lung diseases. Further research is needed to understand the role of chitotriosidase in the pathogenesis of sarcoidosis and its involvement in fibrotic remodelling in certain diffuse lung diseases.
authors.affiliation: Respiratory Diseases Section, Department of Clinical Medicine and Immunology, Siena University, Italy. bargagli2@unisi.it
authors.forename: Elena
authors.initials: E
authors.lastname: Bargagli
authors.forename: Maria
authors.initials: M
authors.lastname: Margollicci
authors.forename: Alice
authors.initials: A
authors.lastname: Luddi
authors.forename: Nicolaos
authors.initials: N
authors.lastname: Nikiforakis
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Perari
authors.forename: Salvatore
authors.initials: S
authors.lastname: Grosso
authors.forename: Anna
authors.initials: A
authors.lastname: Perrone
authors.forename: Paola
authors.initials: P
authors.lastname: Rottoli
mesh_terms: D015415:Biomarkers / Q000032:analysis / Q000378:metabolism; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry*; D005260:Female; D006596:Hexosaminidases / Q000378:metabolism*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000201:enzymology*; D008297:Male; D008875:Middle Aged; D017565:Sarcoidosis, Pulmonary / Q000201:enzymology
chemical_list: D015415:Biomarkers; D006596:Hexosaminidases; C086400:chitotriosidase