Abstract
Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc).
SSc-ILD adversely impacts quality of life and is currently the leading cause of death
in this multisystem disease. Identifying clinically significant SSc-ILD is critically
important. Accurate staging and prognostication remain difficult; however, significant
advances have been made in the last decade. Evidence supports the need to treat patients
with extensive and/or progressive SSc-ILD, while only a subset of patients with limited
ILD may require treatment. Research is urgently required to allow improved prediction
of patients at risk of ILD progression at an early point in the disease, and ideally
prior to its onset, to allow prevention. The last decade has seen the publication
of landmark clinical trials for SSc-ILD. More effective strategies with less toxicity
are under investigation. For those with refractory or very advanced disease, studies
into disease-specific palliative approaches are in their infancy. Lung transplantation
as an option for SSc-ILD remains patient- and center-specific, with data to suggest
equivalent outcomes to other fibrotic lung diseases, in carefully selected cases.
This review aims to provide a comprehensive overview of all key aspects of SSc-ILD.
Keywords
systemic sclerosis - scleroderma - interstitial lung disease - pulmonary fibrosis
