Avancées thérapeutiques dans l’atteinte interstitielle pulmonaire au cours de la sclérodermie systémique

Abstract

Interstitial lung disease complicates systemic sclerosis in about one out of 2 patients. This event is strongly associated with the presence of auto-antibodies anti-topoisomerase type 1. However, it is progressing in a small proportion of the affected patients and several studies are ongoing to try to identify predictive factors. Tools are primarily computed tomography that can stringently depict any interstitial lung changes and also pulmonary functional tests that can identify any functional consequences of these defects. Synthetic immunosuppressants have mostly been offered to affected patients so far despite the weak scientific demonstration of efficacy. If oral cyclophosphamide has been mostly investigated, its high toxicity makes mycophenolate mofetyl frequently the preferred drug as a first line therapy. Criteria to select the patients to be treated remain a matter of debate although simple criteria based on the extent of damages on CT-scan have been published. Targeted therapies are under evaluation with in particular clinical trials assessing tocilizumab and rituximab. Anti-fibrotic drugs are also under development with specifically pirfenidone and nintedanib which are on the market for idiopathic lung fibrosis. The future might be the combination of the above molecules that would appear very complementary taking into account disease pathogenesis.