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Extended report
A vascular endothelial growth factor deficiency characterises scleroderma lung disease
  1. Maria De Santis1,
  2. Silvia Laura Bosello1,
  3. Ettore Capoluongo2,
  4. Rosanna Inzitari2,
  5. Giusy Peluso1,
  6. Paola Lulli2,
  7. Gaetano Zizzo1,
  8. Mario Bocci1,
  9. Barbara Tolusso1,
  10. Cecilia Zuppi2,
  11. Massimo Castagnola2,
  12. Gianfranco Ferraccioli1
  1. 1Institute of Rheumatology and Affine Sciences, Catholic University, Rome, Italy
  2. 2Institute of Biochemistry and Clinical Biochemistry, Catholic University, Rome, Italy
  1. Correspondence to Gianfranco Ferraccioli, Catholic University, Institute of Rheumatology and Affine Sciences, Rome 00168, Italy; gf.ferraccioli{at}


Objectives 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.

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  • Funding The ASRALES Foundation and GILS supported the study.

  • Competing interests None.

  • Patient Consent Obtained.

  • Ethics approval Ethics approval was obtained from the Catholic University ethics committee.

  • Provenance and peer review Not commissioned; externally peer reviewed.