title: A vascular endothelial growth factor deficiency characterises scleroderma lung disease.
abstract: OBJECTIVE
  Vascular endothelial growth factor (VEGF) is thought to play an important role in systemic sclerosis (SSc) pathogenesis. It was found to be upregulated in the serum and in the affected skin of scleroderma patients. However, its involvement in scleroderma lung disease is not clear. This study aimed to evaluate VEGF concentration in the bronchoalveolar lavage fluid (BALF) of scleroderma patients with interstitial lung disease, to correlate the cytokine levels in plasma and in the lung with pulmonary functional, radiological and cellular parameters, and with the progression of lung disease.
  
  
  METHODS
  BALF and plasma VEGF concentrations were analysed by ELISA in 55 SSc patients with lung disease and 17 controls. Cytokine real-time PCR messenger RNA expression in alveolar macrophages was assessed. Lung involvement progression was evaluated after a 1-year follow-up.
  
  
  RESULTS
  VEGF was found to be significantly lower in the BALF of scleroderma patients compared with controls. The lowest concentrations were observed in SSc patients with alveolitis. A decreased VEGF expression in alveolar macrophages was found in SSc patients with alveolitis. VEGF concentration in BALF correlated inversely with the ground glass score on high-resolution CT and with BALF neutrophil cell count. Moreover, SSc patients with a lower VEGF concentration showed a worsening in the interstitial score at follow-up.
  
  
  CONCLUSIONS
  Scleroderma interstitial lung disease is characterised by a VEGF deficiency. Lower concentrations were found in patients with progression of lung disease.
authors.affiliation: Correspondence to Gianfranco Ferraccioli, Catholic University, Institute of Rheumatology and Affine Sciences, Rome 00168, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: De Santis
authors.forename: Silvia Laura
authors.initials: SL
authors.lastname: Bosello
authors.forename: Ettore
authors.initials: E
authors.lastname: Capoluongo
authors.forename: Rosanna
authors.initials: R
authors.lastname: Inzitari
authors.forename: Giusy
authors.initials: G
authors.lastname: Peluso
authors.forename: Paola
authors.initials: P
authors.lastname: Lulli
authors.forename: Gaetano
authors.initials: G
authors.lastname: Zizzo
authors.forename: Mario
authors.initials: M
authors.lastname: Bocci
authors.forename: Barbara
authors.initials: B
authors.lastname: Tolusso
authors.forename: Cecilia
authors.initials: C
authors.lastname: Zuppi
authors.forename: Massimo
authors.initials: M
authors.lastname: Castagnola
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Ferraccioli
mesh_terms: D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D060888:Real-Time Polymerase Chain Reaction; D012595:Scleroderma, Systemic / Q000378:metabolism*; D042461:Vascular Endothelial Growth Factor A / Q000032:analysis / Q000172:deficiency*
chemical_list: D042461:Vascular Endothelial Growth Factor A