Treatment of systemic sclerosis - 27/11/21

Doi : 10.1016/j.lpm.2021.104088

Benjamin Thoreau, MD-MSc ^a,^b, Benjamin Chaigne, MD-PhD ^a,^b, Arthur Renaud, MD-MSc ^a,^c, Luc Mouthon, MD-PhD ^a,^b,^⁎
^a Institut Cochin, INSERM U1016, CNRS UMR 8104, Université de Paris
^b Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
^c Service de Médecine Interne, Centre de Compétence Maladies Systémiques Autoimmunes Rares, CHU de Nantes, Nantes, France

^⁎Corresponding author at:

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Summary

Systemic sclerosis (SSc) is a rare connective tissue disease characterized by skin and visceral fibrosis, vascular hyperreactivity and obliterative vasculopathy. Some of its complications such as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH) and heart involvement can be life-threatening and are associated with a high mortality and a poor prognosis. Many clinical trials were carried out in order to improve the survival and prognosis of SSc patients. The management of SSc is based on the frequent and regular assessment of the potential organ damage, and if present, the establishment of graduated pharmacological therapeutic strategies, associated with non-pharmacological procedures. Several randomized clinical trials have showed significant positive outcomes regarding some specific involvements. Many advances have been made, especially in the field of targeted therapies and personalized medicine, based on specific characteristics of the patient and the SSc.

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Key words : Systemic sclerosis, Treatments, Immunosuppressants, Targeted therapy