Clinical Assessment of ILD

Interstitial lung disease (ILD) is highly prevalent in systemic sclerosis (SSc) both in the diffuse and limited cutaneous subsets. ILD is associated with high mortality and is the main cause of death in SSc patients. The risk of death is directly associated with the extent of ILD on high-resolution computed tomography (HRCT), with impaired lung function, and with ILD progression. At time of SSc diagnosis, all patients should be screened for ILD using HRCT and lung function tests. The diagnosis of ILD is made on HRCT. Risk stratification for severe and progressive disease at diagnosis is important at time point of ILD diagnosis using multiple methods including HRCT and lung function test. ILD progression is frequent and often assessed by declining lung function, but also increasing respiratory symptoms and HRCT changes. Monitoring of all SSc patients with ILD is therefore necessary and needs individually tailored approaches. Monitoring should use multiple methods, including lung function test, 6-minute walking test with oxygen saturation assessment, mapping of respiratory symptoms, and consideration of serial HRCT.