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This study was performed to investigate the role of neutrophil-to-lymphocyte ratio (NLR) in the diagnosis of adult onset Still disease (AOSD) and its performance to improve the sensitivity of the classifications criteria (Yamaguchi and Fautrel Classifications). We conducted a multicenter prospective nationwide case-control study in Internal medicine, Rheumatology and Infectious disease departments, to include successively patients with suspected AOSD (2 or more major criteria of Yamaguchi or Fautrel classifications). All clinical and biological features were collected in a consensual and standardized clinical assessment at baseline and during follow-up. A receiving operating characteristic (ROC) curve was used to reassess the cutoff value of NLR. After determination of the cutoff value for NLR by ROC curve, 2 composite sets (Yamaguchi classification + NLR as a major criterion and Fautrel classification + NLR as a major criterion) were performed and evaluated. One hundred sixty patients were included, 80 patients with AOSD and 60 controls with different diagnoses. Twenty patients with incomplete data were excluded. The cutoff value for NLR equals 4 (area under the curve, AUC: 0.82). The NLR was ≥ 4 in 93.7% (75/80) of AOSD patients with a sensitivity of 93.8% and specificity of 61.7%. The association of NLR as a major criterion with the classification of Yamaguchi or Fautrel improved their sensitivity, respectively for Fautrel (76.3% to 92.5%, P = .004) and Yamaguchi (78.8% to 90%, P = .05). This study validates the NLR as a good simple biomarker of AOSD with a cutoff value of 4 and high sensitivity (93.8%). The addition of NLR (NLR ≥ 4) as a major criterion to the classifications (Yamaguchi and Fautrel) improved significantly their sensitivity and accuracy.
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http://dx.doi.org/10.1097/MD.0000000000029970 | DOI Listing |
BMJ Case Rep
July 2025
Medicine, NYU Langone Health, New York, New York, USA.
Adult-onset Still's disease is a rare systemic autoinflammatory condition of unknown aetiology. It is a diagnosis of exclusion based primarily on clinical features and non-specific laboratory findings using either the Yamaguchi or Fautrel criteria. Here, we present a case of atypical adult-onset Still's disease in a young man with twice-daily fevers, night sweats and evanescent rash on his face but not enough features to meet the Yamaguchi or Fautrel criteria after extensive (but unrevealing) infectious, autoimmune and malignancy workups.
View Article and Find Full Text PDFMed Trop Sante Int
March 2025
Service de médecine interne, CHU de Libreville, BP 2228, Libreville, Gabon.
Introduction: Introduction. Still's Disease (SD) is a rare systemic inflammatory disorder that is most common in children. In the adult form, it can be primary or the resurgence of an infantile form.
View Article and Find Full Text PDFCureus
November 2024
Diabetes and Endocrinology, United Lincolnshire Hospitals NHS Trust, Boston, GBR.
Adult-onset Still's disease (AOSD) is an uncommon systemic inflammatory disorder that presents with diverse, overlapping symptoms, complicating the diagnostic process due to its nonspecific clinical features and the absence of a definitive diagnostic test. Diagnosis is often challenging and relies on excluding other conditions while maintaining a high index of suspicion, supported by specific diagnostic criteria such as Yamaguchi or Fautrel. Prompt recognition and a multidisciplinary approach are essential, as AOSD can progress to life-threatening multiorgan dysfunction due to a hyperinflammatory response.
View Article and Find Full Text PDFFront Immunol
November 2024
Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain.
Objective: Next-generation sequencing (NGS) panels are increasingly used for the diagnosis of monogenic systemic autoinflammatory diseases (SAIDs). However, their role in patients with adult-onset Still's disease (AOSD) remains unknown. This study aims to assess the usefulness of NGS panels in AOSD patients to improve diagnosis and management of the disease.
View Article and Find Full Text PDFMediterr J Rheumatol
March 2024
Department of Medicine, White Plains Hospital, White Plains, NY, USA.
Aim: We have summarised the existing evidence supporting the concept that systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are part of the same Still's disease spectrum.
Methods: A PubMed/Embase database search was conducted using specific search strings and free text words to screen for relevant articles. The search was limited to studies in humans, published up to June 2023, in English-language.