Efficacy of Cyclophosphamide in the Treatment of Interstitial Lung Disease Associated with Systemic Sclerosis

Espinosa, Gerard; Pilar Simeon, Carmen; Angel Plasin, Miguel; Xaubet, Antoni; Muñoz, Xavier; Fonollosa, Vicent; Cervera, Ricard; Vilardell, Miquel

doi:10.1016/S1579-2129(11)70059-3

Article information

Abstract

Background

Cyclophosphamide (CYC) stabilizes the parameters of lung function tests (LFT) of patients with systemic sclerosis (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 (DLCO).

Results

The differences between FVC and DLCO values performed at baseline and those performed at 6, 12, and 24 months were less than 10%, which meant that CYC stabilized functional parameters. There were no differences in FVC or DLCO when patients treated for 6 months were evaluated according to the type of SSc skin involvement of (diffuse or limited) or according to the evolution time of ILD before the start of treatment. 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 lung function parameters in long-term treatment.

Keywords:

Interstitial lung disease

Systemic sclerosis

Cyclophosphamide

Treatment

Resumen

Introducción

La ciclofosfamida (CFM) estabiliza los parámetros del estudio funcional respiratorio (EFR) de los pacientes con esclerosis sistémica (ES) y enfermedad pulmonar intersticial (EPI) tratados durante 12 meses. Existe poca información acerca del tratamiento a largo plazo (24 meses). El objetivo del estudio es analizar el efecto de la CFM endovenosa en los parámetros del EFR de los pacientes con ES y EPI tratados durante 24 meses.

Pacientes y método

Estudio retrospectivo de 37 pacientes con EPI asociada a esclerodermia, tratados con CFM endovenosa durante 24 meses y evaluados de forma periódica mediante EFR (basal, a los 6, 12 y 24 meses). En este se evaluó la capacidad vital forzada (FVC) y la capacidad de transferencia de monóxido de carbono (DLCO).

Resultados

Las diferencias entre los valores de FVC y DLCO basales y los realizados a los 6, 12 y 24 meses fueron menores del 10%, lo que significa que la CFM estabilizó los parámetros funcionales. Tampoco se detectaron diferencias en la FVC ni en la DLCO cuando se valoró a los pacientes tratados durante 6 meses de acuerdo al tipo de afectación cutánea de la ES (difusa o limitada), o según el tiempo de evolución de la EPI antes del inicio del tratamiento. Si bien los pacientes con restricción grave (FVC < 70%) al inicio mostraron mayor mejoría, esta fue en todos los casos inferior al 10%.

Conclusión

En esta serie de pacientes con EPI asociada a ES, la CFM endovenosa estabilizó los parámetros funcionales respiratorios en el tratamiento a largo plazo.

Palabras clave:

Enfermedad pulmonar intersticial

Esclerosis sistémica

Ciclofosfamida

Tratamiento

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