Characteristics and Clinical Value of 18F-FDG PET/CT in the Management of Adult-Onset Still's Disease: 35 Cases.

Josselin Brisset,Anne Lemaire,Jacques Monteil,Martin Killian,Eric Liozon,Anne-Laure Fauchais,Kim Heang Ly,Yvan Jamilloux,Stephanie Dumonteil,Tomasz Chroboczek,Pascal Sève,Mathieu Gerfaud-Valentin,Guillaume Lades,Guillaume Gondran

doi:10.3390/jcm10112489

Abstract

While the diagnosis of adult-onset Still’s disease (AOSD) involves the exclusion of differential diagnoses, the characteristics and value of 18F-Fluorodeoxyglucose (18F-FDG) Positron Emission Tomography coupled with CT (PET/CT) in the management of AOSD remain poorly known. Our retrospective study included patients from four centers, fulfilling Yamaguchi or Fautrel criteria, who underwent a PET/CT during an active AOSD. Thirty-five patients were included. At the time of PET/CT, the Yamaguchi criteria were met in 23 of 29 evaluable cases. PET/CT showed bone marrow (74.3%), lymph node (74.3%), and splenic (48.6%) FDG uptake. Despite arthralgia or arthritis in most patients, joints were rarely the sites of 18F-FDG accumulation. The spatial distribution of 18F-FDG uptake was nonspecific, and its intensity could be similar to malignant disease. Lymph node or bone marrow biopsy was performed after PET/CT in 20 patients (57.1%). The intensity of bone marrow; splenic and lymph node hypermetabolism appeared to be correlated with disease activity. Abnormal PET/CT in the cervical lymph nodes and age ≥ 60 years seemed to be predictive factors for monocyclic evolution. The clinical value of PET/CT is not in direct diagnosis; but as an aid in excluding differential diagnoses by searching for their scintigraphic features and guiding biopsy.

Highlights

Adult-onset Still’s disease (AOSD) is a rare systemic multigenic autoinflammatory disorder, the pathogenesis of which remains poorly understood [1,2,3]
The following data were collected using a standardized form: (1) clinical features: all manifestations observed in the flare during which 18F-FDG Positron Emission Tomography coupled with computed tomography (CT) (PET/CT) was performed; (2) imaging features with a detailed description of 18F-FDG PET/CT results; (3) laboratory parameters measured at the time of disease flare, as close as possible to the time of 18F-FDG PET/CT when several data were available; (4) clinical course and treatments
Lymph node or bone marrow biopsies were performed after the PET/CT in most cases in our study

Summary

Introduction

Adult-onset Still’s disease (AOSD) is a rare systemic multigenic autoinflammatory disorder, the pathogenesis of which remains poorly understood [1,2,3]. It is characterized by high spiking fever with transient skin rash, arthralgia or arthritis, sore throat, lymphadenopathy, hepatosplenomegaly, neutrophilic leukocytosis, hyperferritinemia with low glycosylated ferritin (

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