Newer insights into the management of interstitial lung disease in systemic sclerosis

Abstract

Interstitial lung disease (ILD) is a debilitating complication of systemic sclerosis (SSc) and now the leading cause of death in SSc patients, largely from progressive respiratory failure or advanced pulmonary hypertension. Despite significant advances in our understanding of the epidemiology and pathogenesis of SSc-ILD, there are significant uncertainties in the approach to managing these patients given the heterogeneity of presentation, substantial variability in progression, and presence of comorbid cardiopulmonary conditions, particularly pulmonary hypertension and esophageal dilation with recurrent aspiration pneumonitis that portend poor prognosis. Early detection of progressive lung involvement based on worsening pulmonary physiology and quantification of fibrosing alveolitis severity on high-resolution computed tomography is critical as response to immunomodulatory agents is usually best when initiated earlier in the disease course. A selected group of patients may benefit from early referral for hematopoietic stem cell transplantation or lung/heart–lung transplant. The last decade has seen a significant advance in evidence-based approaches to treatment of SSc-ILD with immune suppressants, and there are several ongoing treatment trials with recent advances in understanding of the role of pro-inflammatory and profibrotic cytokines in SSc-ILD. The efficacy of antifibrotic agents in idiopathic pulmonary fibrosis has also provided another promising avenue for utilization in these patients. In this review, we will provide an up-to-date review of the treatment options for SSc-ILD, the ongoing studies moving this field forward, emerging treatments for SSc-ILD, and propose a management algorithm for SSc-ILD, based on the available evidence in the literature and our experience.