Abstract

Objective: The objective of the study was to assess the advantages of 18F-fluorodeoxyglucose (FDG) positron emission tomography and computed tomography with magnetic resonance (PET/CT-MR) in diagnosing and monitoring patients with adult-onset Still's disease (AOSD).Methods: Participants in this retrospective case-control study underwent whole-body 18F-FDG-PET/CT-MR imaging. All PET scans were qualitatively and semiquantitatively analyzed using standardized uptake values (SUVs) normalized to liver uptake, i.e., we calculated the ratio (SUVr) between the minimum, maximum, and mean SUVs for different organs and tissues and the mean SUV for the liver. Disease activity scores were assessed using Pouchot's criteria.Results: Eighteen patients diagnosed with AOSD and 24 controls (non-AOSD patients diagnosed with solid tumors, excluding lymphomas) were considered. A total of 38 PET/MR and nine PET/CT scans were analyzed. AOSD patients had higher SUVr than controls. All SUVr differed significantly between the patient and control group for bone marrow, and for the spleen, the only difference lacking statistical significance concerned the ratio of the minimum SUV for spleen to the mean SUV for liver. Though limited in number, AOSD responders to therapy showed lower uptakes during the period monitored. No correlations were found between Pouchot's scores and SUVr.Conclusion: Our data revealed higher spleen and bone marrow 18F-FDG uptakes on PET/CT and PET/MR images in AOSD patients than in controls. Together with clinical examinations and laboratory data, PET/CT and PET/MR seemed more reliable than Pouchot's score in assessing disease activity.

Highlights

  • With an incidence of 0.16–0.62 per 100,000 population worldwide, adult-onset Still’s disease (AOSD) is a rare multisystem inflammatory disease that usually affects young adults [1]

  • We analyzed 14 18F-FDG positron emission tomography with magnetic resonance (PET/MR) and 9 18F-FDG positron emission tomography and computed tomography (PET/CT) performed in the AOSD group, and 24 18F-FDG PET/MRs performed in the control group

  • Analyzing our study data confirmed that AOSD patients had a higher 18F-FDG uptake on PET/CT-MR images than the controls

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Summary

Introduction

With an incidence of 0.16–0.62 per 100,000 population worldwide, adult-onset Still’s disease (AOSD) is a rare multisystem inflammatory disease that usually affects young adults [1]. The exact pathogenesis behind AOSD remains unknown, a combination of genetic and environmental factors seems to be involved [2]. AOSD is clinically characterized by daily high-spiking fever, evanescent maculopapular skin rash, arthritis, musculoskeletal involvement, sore throat, and hepatosplenomegaly [3]. Cardiopulmonary manifestations and liver dysfunction are rare. Typical laboratory findings include leukocytosis with neutrophilia, hyperferritinemia, high transaminases, and elevated acute-phase reactants, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Fautrel et al proposed glycosylated ferritin (GF) as a diagnostic biomarker of AOSD because low concentrations have been noted (

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