Pneumopathie infiltrante diffuse de la sclérodermie systémique

Abstract

Interstitial lung diseases (ILD) associated with systemic sclerosis (SSc) are mainly encountered in patients with diffuse disease, although they may also be seen in patients with limited cutaneous SSc. ILD screening must be performed regularly, with high-resolution computed tomography and pulmonary function tests (TLCO). Up to 75% of patients with diffuse SSc develop a form of ILD. ILD remains stable in most patients and does not worsen. The nonspecific nature of SSc-associated ILD makes it different from idiopathic ILD and helps to explain its better prognosis. Nonetheless, ILD is one of the two leading causes of death in SSc patients. Treatment of SSc-associated ILD is not yet well codified. Antifibrotic treatments have not proved beneficial, and the efficacy of cyclophosphamide, which has been used to treat this condition for 15 years, has been shown to be very limited against SSc-associated ILD. A subgroup of patients with rapidly progressive ILD might benefit from intravenous cyclophosphamide pulses in association with 15 mg/d prednisone.