Scleroderma-ILD

Systemic sclerosis is a clinically heterogeneous multisystemic disease with internal organ complications including interstitial lung disease. Interstitial lung abnormalities are evident on HRCT Chest in up to 80% of patients and just under half develop clinically significant ILD which is the leading cause of mortality in SSc. Patient stratification in SSc-ILD based on clinical and laboratory characteristics is invaluable to identify those most at risk for developing progressive ILD from those more likely to have a mild disease or a slow progressive trajectory. Baseline HRCT and lung function with regular lung function monitoring are key to effective management. Detailed analyses from recent well-conducted clinical trials have confirmed prognostic markers of lung function decline and predictive markers of response thus inform subgroups of patients likely to benefit from therapy. Data from real world experience seek to confirm results from these highly selective clinical trial populations. Considering the increasing therapeutic options for SSc-ILD coupled with the systemic nature of SSc, shared management between respiratory physicians and rheumatologists is key