Background Systemic sclerosis (SScl) is a chronic inflammatory autoimmune rheumatic disease with a UK prevalence of 2–10 per 100,000. It is a heterogenous disease characterised by varying degrees of dermal and organ fibrosis. Interstitial lung disease (ILD) occurs in 60–80% of patients and ranges from mild, clinically trivial disease to extensive fibrosis that results in respiratory failure and premature death. Therapeutic options include cyclophosphamide, mycophenylate mofetil and rituximab. Clinical decisions are complex and decisions to treat or not have historically been based on radiology and lung function tests, neither of which (at a single time point) give a dynamic view of disease progression. Novel biomarkers are urgently needed to predict disease activity, progression and response to treatment in patients with SScl-ILD.
Aims To investigate the potential of 18Fluoro-deoxyglucose Positron Emission Tomography (FDG-PET)/CT to act as a prognostic and response biomarker in patients with SScl-ILD.
Methods 35 SScl-ILD patients were prospectively recruited for 18F-FDG-PET/CT. Patients were screened for lung involvement using clinical assessment, chest X-ray and pulmonary function testing (PFT). Those with confirmed SScl-ILD underwent combined high resolution CT scan (HRCT)/PET scanning. The imaging signal and clinical findings were correlated with the need for, and response to, therapy. Follow up was with clinical assessment, PFT and when a change in treatment was indicated, repeat imaging.
Results The overall maximum pulmonary uptake of 18F-FDG (SUVmax), the minimum pulmonary uptake or background-lung-activity (SUVmin) and target-to-background (SUVmax/SUVmin) ratio (TBR) were quantified using routine region-of-interest analysis. Kaplan-Meir analysis was used to identify associations with disease progression and response to treatment
Conclusions We have shown that high pulmonary uptake of 18F-FDG is associated with disease activity and progression in patients with SScl-ILD. These PET findings can be used to give additional information, supplemental to PFTs, which may then aid clinical treatment decisions.