TY - JOUR
T1 - Oral cyclophosphamide improves pulmonary function in scleroderma patients with fibrosing alveolitis
T2 - Experience in one centre
AU - Beretta, Lorenzo
AU - Caronni, Monica
AU - Raimondi, Massimo
AU - Ponti, Alessandra
AU - Viscuso, Tiziana
AU - Origgi, Laura
AU - Scorza, Raffaella
PY - 2007/2
Y1 - 2007/2
N2 - Lung involvement constitutes nowadays the major cause of morbidity and mortality in scleroderma patients. Pulmonary fibrosis in systemic sclerosis (SSc) is thought to be the consequence of interstitial inflammation. Early diagnosis and treatment of active alveolitis is essential to prevent the deterioration of pulmonary function, improving outcome in SSc patients. The aim of the study was to investigate the effect of 1-year treatment with oral cyclophosphamide (CYC) on the evolution of interstitial lung disease in scleroderma patients with a diagnosis of active alveolitis. An open-label one-arm monocenteric study was conducted on 33 scleroderma patients with active alveolitis - defined as the presence of areas of 'ground-glass attenuation' on high-resolution computed tomography and a recent deterioration in lung function-treated with oral CYC 2 mg kg-1 day-1 for 1 year and medium-low dose steroids (prednisone 25 mg for 3 months and then tapered to 5 mg/day). Results showed that diffusing capacity for carbon monoxide (DLco) values remained stable after 6 months of treatment and significantly increased after 12 months (2.06±1.38, 2.21±1.62 and 2.39±1.64 mmol/min/kPa, at baseline/6/12 months, respectively; p
AB - Lung involvement constitutes nowadays the major cause of morbidity and mortality in scleroderma patients. Pulmonary fibrosis in systemic sclerosis (SSc) is thought to be the consequence of interstitial inflammation. Early diagnosis and treatment of active alveolitis is essential to prevent the deterioration of pulmonary function, improving outcome in SSc patients. The aim of the study was to investigate the effect of 1-year treatment with oral cyclophosphamide (CYC) on the evolution of interstitial lung disease in scleroderma patients with a diagnosis of active alveolitis. An open-label one-arm monocenteric study was conducted on 33 scleroderma patients with active alveolitis - defined as the presence of areas of 'ground-glass attenuation' on high-resolution computed tomography and a recent deterioration in lung function-treated with oral CYC 2 mg kg-1 day-1 for 1 year and medium-low dose steroids (prednisone 25 mg for 3 months and then tapered to 5 mg/day). Results showed that diffusing capacity for carbon monoxide (DLco) values remained stable after 6 months of treatment and significantly increased after 12 months (2.06±1.38, 2.21±1.62 and 2.39±1.64 mmol/min/kPa, at baseline/6/12 months, respectively; p
KW - Cylophosphamide
KW - Fibrosingal veolitis
KW - HRCT
KW - Systemics clerosis
UR - http://www.scopus.com/inward/record.url?scp=33845867399&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33845867399&partnerID=8YFLogxK
U2 - 10.1007/s10067-006-0254-x
DO - 10.1007/s10067-006-0254-x
M3 - Article
C2 - 16614793
AN - SCOPUS:33845867399
VL - 26
SP - 168
EP - 172
JO - Clinical Rheumatology
JF - Clinical Rheumatology
SN - 0770-3198
IS - 2
ER -