Evaluating the multivisceral involvement on adult-onset Still’s disease to retrieve imaging-based differences in patients with and without macrophage activation syndrome: results from a single-centre observational study

In this study, we aimed at describing the multivisceral involvement on adult-onset Still's disease (AOSD) to retrieve imaging-based differences in patients with and without macrophage activation syndrome (MAS). From our historical cohort, patients were assessed among those who underwent a total body CT scan. Clinical and CT scan characteristics of AOSD patients with and without MAS were compared. Out of 39 AOSD patients evaluated, 14 were complicated by MAS. These patients showed higher values of ferritin and systemic score. AOSD patients with MAS presented a higher prevalence of lung disease, hepatomegaly, splenomegaly, abdominal effusions, and lymph node enlargement than others without this complication. In addition, the presence of these manifestations significantly correlated with the systemic score, furtherly reinforcing its prognostic value. Due to the specific design of our study, our findings could be burdened by a selection bias since assessing those patients underwent a total body CT scan. Thus, these data should be prudently generalised suggesting the need of further studies to fully elucidate this issue. Our findings showed a higher prevalence of multiorgan involvement in AOSD patients with MAS, suggesting imaging-based differences, although other studies are needed to fully assess this issue. Pulmonary disease, hepatomegaly, splenomegaly, lymph node enlargement, and abdominal effusions were associated with a more aggressive subset of AOSD. Key Points The importance of an accurate assessment AOSD multivisceral involvement is suggested since it is associated with life-threatening complications. A higher prevalence of multiorgan involvement in AOSD patients with MAS could be recognised, than others without this complication, suggesting imaging-based differences. AOSD multivisceral involvement may correlate with the systemic score, furtherly reinforcing its prognostic value.