Proposal of a new diagnostic algorithm for adult-onset Still’s disease

Daghor-Abbaci, K.; Hamadouche, Nadia Ait; Dahou-Makhloufi, C.; Mechid, F.; Otmani, F.; Makrelouf, Mohamed; Otmane, Amel; Smail, N.; Boucelma, Malika; Aissat, Fatma Zohra; Lefkir-Teffiani, Salima; Bengana, Bilel; Tebaibia, Amar; Taharbouchet, Baya; Ayoub, Soraya; Benziane, Brahim; Oumnia, N.; Haouichet, Chafika; Hanni, Fella; Laraba, N.; Hakem, Djennete; Benfenatki, N.; Berrah, A.

doi:10.1007/s10067-023-06509-8

Cited by 6 publications

(2 citation statements)

References 46 publications

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“…Kontzias and Efthimiou 49 have proposed a diagnostic algorithm ( Figure 2 ) that emphasises that atypical presentations may occur in AOSD and that symptoms such as a high spiking fever, evanescent rash, and arthritis need not be present concomitantly at the onset of the disease. Recently, an algorithm with a points-based score (7 points), with a sensitivity of 92.5% and a specificity of 93.3%, was proposed by Daghor-Abbaci et al 80 Patients eligible for this algorithm should have at least two of the following criteria: fever ≥ 39°C, arthralgia or arthritis, pharyngitis, neutrophils percentage ≥80% or NLR ≥ 4, or ferritin > N.…”

Section: Still’s Disease Continuummentioning

confidence: 99%

Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as Part of the Still’s Disease Continuum

Kontzias,

Petryna,

Nakasato

et al. 2024

MJR

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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. Summary: sJIA and AOSD are rare autoinflammatory disorders that have similar pathophysiological and clinical features. The clinical presentations of sJIA and AOSD are highly variable, with differential diagnoses that include a broad range of malignancies, infectious diseases, and autoimmune disorders, which contribute to delays in diagnosis. Several sets of classification exist to help diagnose patients in clinical practice; the International League of Associations for Rheumatology criteria for sJIA and the Yamaguchi and Fautrel criteria for AOSD are the most-used criteria. The therapeutic strategy for Still’s disease aims to relieve signs and symptoms, prevent irreversible joint damage and potentially life-threatening complications, and avoid deleterious side effects of treatment. Recently, targeted therapies such as interleukin (IL)-1 and IL-6 inhibitors have become available for the treatment of sJIA and AOSD. While these biologics were originally largely reserved for patients in whom non-steroidal anti-inflammatory drugs, corticosteroids and conventional synthetic disease-modifying anti-rheumatic drugs had failed, they are increasingly used earlier in the treatment paradigm. Among IL-1 inhibitors, canakinumab is the only biologic approved in the US for the treatment of both sJIA and AOSD.

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Section: Still’s Disease Continuummentioning

confidence: 99%

Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as Part of the Still’s Disease Continuum

Kontzias,

Petryna,

Nakasato

et al. 2024

MJR

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“…Several sets of classification criteria have been proposed thus far, with Yamaguchi's and Fautrel's being the most employed [10,11], however, at present, there are no diagnostic criteria available. To facilitate the diagnostic process, a new algorithm fixed on points-based score has been recently proposed, aiming to be an appropriate diagnostic tool for clinical practice and research [12]. The clinimetric score known as modified Pouchot's score (mPS) [13], is uncommonly employed to evaluate disease activity, however, a recent study comparing the mPS and the systemic manifestation score (mSMS), demonstrated that both mSMS and mPS can assess disease severity, with a higher discriminative ability of mPS to identify patients with high disease severity compared with mSMS.…”

Section: Introduction: Clinical Aspects Of a Unique Diseasementioning

confidence: 99%

Adult-Onset Still’s Disease (AOSD): Advances in Understanding Pathophysiology, Genetics and Emerging Treatment Options

Bindoli,

Baggio,

Doria

et al. 2024

Drugs

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Adult-onset Still’s disease (AOSD) is a multisystemic complex disorder clinically characterised by episodes of spiking fever, evanescent rash, polyarthritis or diffuse arthralgias; multiorgan involvement may develop according to the hyper-inflammatory extent. The pathogenesis of AOSD is not completely recognised. The central role of macrophage activation, which results in T helper 1 (Th1) cell cytokine activation, is well established. Pro-inflammatory cytokines such as interleukin (IL)-1, IL-6 and IL-18 play a fundamental role in disease onset and progression. The disease may develop in both children and adults with overlapping clinical features, and although several subsets depending on the clinical manifestations and the cytokines expressed have been identified, the dichotomy between systemic juvenile idiopathic arthritis (sJIA) and AOSD nowadays has been overcome, and the pathology is considered a disease continuum between ages. Various therapeutic approaches have been evaluated thus far, and different compounds are under assessment for AOSD treatment. Historically, glucocorticoids have been employed for treating systemic manifestations of Still’s disease, while conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) demonstrated efficacy in controlling the articular manifestations. Currently, biological (b) DMARDs are widely employed; IL-1 inhibitors such as anakinra and canakinumab have proven to have high efficacy and an excellent safety profile and the anti-IL-6 tocilizumab is approved for sJIA, with several trials and longitudinal studies confirming its efficacy and safety. Moreover, in the light of the ‘window of opportunity’, new evidence showed that the earlier these treatments are initiated, the sooner clinical inactivity can be achieved. Other treatment options are being considered since several molecules involved in the disease pathophysiology can be targeted through various mechanisms. This review will provide a broad overview of AOSD pathophysiology, insights into specific organ manifestations and the currently available treatments with the identification of potential therapeutic targets involved in AOSD pathogenesis will be outlined.

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Diagnostic algorithm of adult-onset Still’s disease—unmet need and problems with methodology

Băicuș

2023

Clin Rheumatol

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Proposal of a new diagnostic algorithm for adult-onset Still’s disease

Cited by 6 publications

References 46 publications

Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as Part of the Still’s Disease Continuum

Diagnosing and Treating Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease as Part of the Still’s Disease Continuum

Adult-Onset Still’s Disease (AOSD): Advances in Understanding Pathophysiology, Genetics and Emerging Treatment Options

Diagnostic algorithm of adult-onset Still’s disease—unmet need and problems with methodology

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