Eficacia de la ciclofosfamida endovenosa en el tratamiento de la enfermedad pulmonar intersticial asociada a la esclerosis sistémica

Abstract

Background Cyclophosphamide (CYC) stabilizes the parameters of lung function tests (LFT) of patients with (SSc) and interstitial lung disease (ILD) treated for 12 months. There is little information about long-term treatment (24 months). The aim of this study is to analyze the effect of intravenous CYC in LFT parameters in patients with SSc and ILD treated for 24 months. Patients and method Retrospective study of 37 patients with ILD associated with scleroderma treated with intravenous CYC for 24 months and regularly assessed by LFT (at baseline, 6, 12 and 24 months) including forced vital capacity (FVC) and transfer capacity of carbon monoxide (DL CO). To evaluate response to treatment the recommendations of the ATS and SEPAR were considered. Results The difference between FVC and DL CO values performed at baseline and those performed at 6, 12, and 24 months were less than 10%, which meant that CYC stabilized LFT. There were no differences in LFT when patients treated for 6 months were evaluated according to the type of skin involvement of the SSc (diffuse or limited) and the duration of the ILD. Although patients with severe restriction (FVC<70%) showed more improvement, it was less than 10% in all cases. Conclusion In this series of patients with ILD associated with SSc intravenous CYC was effective in stabilizing LFT in long-term treatment.