Introduction

Interstitial lung disease (ILD) is common in patients with systemic sclerosis (SSc). Currently nintedanib is the only approved treatment which has demonstrated a reduction in lung function decline in those with evidence of progressive fibrosing interstitial lung disease (PF-ILD).  

Aim

The aim of this retrospective study in a large regional centre for SSc was to ascertain common factors in patients with systemic sclerosis associated with interstitial lung disease (SSc-ILD) as well as variables which predisposed SSc-ILD patients to develop PF-ILD.

Methods

717 patients were identified with evidence of active SSc seen in the last 5 years. These patients were further phenotyped to identify those with radiographic evidence of ILD and PF-ILD as defined by progressive symptoms, FVC decline and radiological progression. Standard demographics and autoimmune markers were also collected to ascertain any common variables predisoposing a SSc patinet to ILD.  

Results

178 patients were identified to have evidence of SSc-ILD of these patients a fifth had evidence of diffuse SSc associated ILD. The most common pattern of ILD was noted was NSIP. Evidence of progressive ILD was noted radiographically and symptomatically in 30% of patients. In those patients with progressive SSc-ILD the radiographic pattern associated with progression was proportionally more in those with a UIP pattern, these patients also had a shorter SSc disease course in comparison. PF-ILD was seen more commonly in those with limited SSc-ILD. 

Conclusion

This single centre study has demonstrated SSc-ILD patients with a more aggresive disease course, UIP pattern fibrosis and comorbidties are predisposed to PF-ILD