Treatment of Interstitial Lung Disease in Systemic Sclerosis

Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) and a major cause of death. The course of SSc-ILD is characterized by a great variability, ranging from stable disease for years to rapidly progressive disease that can lead to respiratory failure and death. There are currently no robust markers to risk-stratify SSc-ILD patients. Prognosis can be assessed both on the basis of baseline severity, but also according to disease progression over time, using a combination of clinical, biological, radiographic, and lung function data. The treatment of ILD in SSc is based on drug therapy and supportive care. Classes of drug treatments include cytotoxic therapies, biological therapies, antifibrotic agents, and lung transplantation. In recent years, numerous clinical trials have been published and great progress has been made in developing effective treatment options for SSc-ILD. These positive results have led to the approval by drug agencies of the first drugs for SSc-ILD, Nintedanib and Tocilizumab. In this review, we discuss criteria for guiding treatment initiation, modalities of follow-up, current treatment options, and potential new therapeutic targets in SSc-ILD. Based on the available evidence, our clinical experience, and recent recommendations, we propose an algorithm to guide the physician in the decision and choice of treatment in clinical practice. This algorithm is based on individual patient stratification according to clinical manifestations (including extra-pulmonary involvement), disease severity, and risk of progression.